Manual of Pediatric Cardiac Care: Volume II

Foreword
Preface
Acknowledgments
Contents
About the Editors
Editor in Chief
Editors
Contributors
A Pledge to the Parents of Children with Congenital Heart Disease
Gaining Trust of the Parents Who Have Loaned Us Their Child for Treatment
Part I: Ethical Considerations in Pediatric Cardiac Patients
	Ethical Considerations and Ethical Decision Making in Pediatric Cardiac Care
		Background: Ethical Encounters of Different Levels of Complexity
		Ethical Decision Making in Diagnosis or Management
		Imparting Bad News to Parents of a Child in Cardiac Care
		Genetic Research Ethics for Cardiac Conditions by Use of a Case Scenario
		References for Further Reading
	Practical Aspects of Ethical Decision Making in CHD
		Background
		Genetic Testing and Screening for CHD
		Correct Diagnosis and Family Counselling
		Challenges with Certain Syndromes
		Decisions for Invasive Interventions: Full Repair Versus Palliative Surgery
		Extraordinary Management Options
		Transition from Pediatric CHD to Adult CHD
		End-of-Life Care
		Summary
		References for Further Reading
	Family Counselling and Communication in Children with Complex CHD
		Background
		General Aspects and Challenges in Children with CHD
		Why Proper Family Counselling Is Extremely Important?
		Benefits of Family Counselling
		Successful Counselling
		To Do’s for the Pediatric Cardiologist After Confirmation of the Diagnosis
		What Are the Most Frequently Asked Questions by Parents?
		What Are the Goals of Genetic Counselling in CHD?
		What About If Cardiac Defects Are Diagnosed During Pregnancy?
		The “Journey” of Counselling Parents with CHD
		Communication in Children with Complex CHD
			Complex CHD with Full Surgical Repair
			Complex CHD with Palliative Strategy
			Complex CHD Without Intervention
		Summary
		References for Further Reading
Part II: Pediatric Cardiac Surgery
	Overview of Pediatric Cardiac Surgery with Historical Timeline and Definitions of Named Surgical Procedures
		Background
		References for Further Reading
	Quality Standards and Measures in Pediatric Cardiac Surgical Procedures
		Background
		Surgical Decision-Making Process (Fig. 1)
		Informed Consent for Surgery
		Intra-Operative Trans-Esophageal Echocardiography (TEE)
		Quality Measures for Cardiac Surgical Procedures
		References for Further Reading
	General Anesthetic Considerations During Pediatric Cardiac Surgical Procedures
		Background
		Preoperative Evaluation and Preparations
			History and Physical Examination
			Review of Diagnostic and Medication Data, as Listed Below, and Anesthesia Consent
		Premedication and Nothing by Mouth (NPO) Orders
		Intraoperative Management
			Induction of Anesthesia
			Maintenance of Anesthesia
			Physiologic Monitoring (Fig. 1)
			Separation from CPB (Fig. 2)
			Bleeding Control
		Post-operative Care
		References for Further Reading
	Cardiopulmonary Bypass
		Background
		Cardiopulmonary Bypass Machine Components (Fig. 1)
		Cardiopulmonary Bypass Connections (Fig. 2)
		Cardiopulmonary Bypass Machine Components (Fig. 3)
		References for Further Reading
	Selected Surgical Procedures for Repair and Palliation in Pediatric Cardiac Surgery
		Background
		Coarctation Repair
		Sinus Venosus Atrial Septal Defect (SVASD)
		Complete Atrioventricular Septal Defect (AVSD)
		Transposition of the Great Arteries (TGA)
		Ross-Konno Procedure
		Single Ventricle Palliation
			Stage I Palliation
			Stage II Palliation
			Stage III Palliation
		Biologic and Synthetic Materials Used in Pediatric Cardiac Surgery
			Patches
			Conduits
			Valves
		Management of Pacing Wires and Chest Tubes after Pediatric Cardiac Surgery
			Pacing Wires
			Chest Tubes
		References for Further Reading
	A Brief Pictorial Overview of the Pioneers in Cardiac Surgery and Pediatric Cardiology
		Background
		References for Further Reading
Part III: Pediatric Cardiac Intensive Care Unit (PCICU) Guidelines
	Admission of Patients in Pediatric Cardiac Intensive Care Unit (PCICU)
		Background
		Admission of Patients from the Operation Room or Other Places
			Priorities Before Arrival to PCICU
			Priorities on Arrival
				On Arrival to PCICU
			Handover
			ICU Physician “Action List”
			Guidelines for Obtaining Echocardiograms
			Routine Drug and Fluid Orders
			Checklist for Admission of Patients from Theatre
		References for Further Reading
	Guidelines for Transport of PCICU Patients Within the Hospital
		Background
		FIRST: Ask the Following Questions
		Preparation
		After Transport
		Additional Advices
		References for Further Reading
	Access and Expert Management of the Airway
		Background
		Basic Emergency Airway Management
			Bag-Mask Ventilation
			Intubation
			Tracheal Tube Sizes
			Sedation and Anesthesia
			Intravenous Access and Drugs Used During Intubation
			The Intubation Sequence
			Rapid Sequence Induction
			Complications
			Long-Term Complications
			Summary of Key Points
		References for Further Reading
	Insertion of Arterial and Central Lines
		Background
		Ultrasound Guidance
		Patient Preparation
		Insertion Technique
		Arterial Line Insertion
			Indications
			Possible Cannulation Sites
			Complications
			Care of Arterial Lines
		Central Venous Catheterization
			Indications
			Relative Contraindications
			After Insertion
		Complications of Central Venous Line Insertion
			Arterial Puncture
			Pneumothorax
			Needle Trauma
			Arrhythmia
			Catheter Malposition
			Air Embolism (Is Preventable)
			Pericardial Tamponade
			Venous Thrombosis
			Catheter-Related Infection
		References for Further Reading
	Ventilation and Respiratory Support
		Background
		Factors Influencing Perioperative Pulmonary Function
		Postoperative Ventilation
			Definition
			Aims of Postoperative Ventilation
		Ventilators
			Conventional
			High Frequency Devices
			How to Set Up a VOLUME CONTROLLED Ventilator
			How to Set Up a PRESSURE-LIMITED Ventilator
			After Connecting a Patient to a Ventilator Always Check That
			Problems with Ventilation
			Positive End-Expiratory Pressure (PEEP)
				CAUTION Is Advised in the Following Circumstances
		Tracheal Tubes
		Weaning of Patients from Mechanical Ventilation
		Extubation
		High-Frequency Oscillatory Ventilation (HFOV)
			Settings
			Setting Modifications/Remarks
			Patients with Open Sternum
			Bronchial Washings/Bronchoalveolar Lavage
		References for Further Reading
	Pediatric High-Flow Nasal Cannula and Non-invasive Ventilator Support
		Background
		What Are the Advantages of Non-invasive Ventilation?
		How Does the High Flow and Non-invasive Ventilation Work?
		What Are the Indications and Contra-indications for Non-invasive Ventilation and High-Flow Nasal Cannula Support?
		Different Interfaces Used in Non-invasive Ventilation (Figs. 1, 2, and 3)
		Starting and Setting Up Non-invasive Ventilation or High Flow
		Complications Associated with Non-invasive Ventilation
		Monitoring of Patient on High Flow or NIV
		Can We Feed Child While on NIV or High-Flow Support?
		Predictor of Responsiveness to NIV or High Flow, Weaning of High Flow and NIV
		Weaning and Discontinuation of NIV or High Flow
		NIV Success
		References for Further Reading
	Pulmonary Hypertensive Crisis in PCICU
		Background
			Monitoring
			Precipitating Factors
			Signs of Pulmonary Hypertension
			Aims of Treatment
			Preventive Measures
			Reduction of Support After Resolution of Crises
		References for Further Reading
	Inhaled Nitric Oxide (iNO)
		Background
			Indications
			iNO Dosage
			Weaning from iNO
			iNO Toxicity
			Administration, Health, and Safety
			NO Administration Systems
			Scavenging of NO and NO2 from the Ventilator Exhaust System
			Suctioning, Physiotherapy and Handbag Ventilation with iNO
			Accidental Discontinuation of iNO
			Summary Checklist
		References for Further Reading
	Extracorporeal Membrane Oxygenation (ECMO) in PCICU
		Background
			How Does ECMO Work?
			Who Is Eligible for ECMO?
			Clinical Applications of ECMO
			Cannulation
			Determining Membrane FiO2, Pump, Sweep Flow
			Ventilator Setting During ECMO
			Criteria for Sufficient Organ Perfusion on ECMO
			Temperature
			Anticoagulation and Blood Products
			Sedation
			Weaning from ECMO
			Example of Set of Order for ECMO Patient
		References for Further Reading
	Management of Postoperative Bleeding
		Background
		Bypass-Induced Coagulation Abnormalities
			When Do We Say That a Child Is Bleeding Post cardiac Surgery?
			What Are the Factors That Influence Our Decision to Give Blood and Blood Products?
			How Should I Monitor Bleeding?
			Decision for Exploration
			Decision for exploration
			Use of Blood and Blood Components in the Management of Postoperative Bleeding
		Cardiac Tamponade
			Action in Suspected Tamponade
		References for Further Reading
	Use of Blood Products in Critically Ill Children with Focus on Cardiac Diseases
		Background
		Red Cell Components
			Estimation of Blood Volume
			Content of Packed Red Blood Cells (PRBC)
			Blood Transfusion Can Be Minimized By
			Recommended PRBC Transfusion Thresholds for Anemic Patients in PCICU
			Types of PRBC’s
			Compatibility
			Dose
			Caution
			Type of Stored Packed Red Blood Cells Components (Table 1)
			Indications for Irradiated PRBC
			Complications
		Platelet Transfusion in Pediatric Critical Care
			Causes of Thrombocytopenia
			Approach to Patients with Decreased Platelets or Dysfunction of Platelets in Critical Care (Fig. 3)
			Indications for of Platelet Transfusions and Minimum Threshold
			Processing of Platelet Transfusions
			Processing of Platelet Transfusions
			Dose of Platelet Transfusions
		Fresh Frozen Plasma (FFP) and Cryoprecipitate Transfusion in Pediatric Critical Care
			Plasma Components
			Indications for Fresh Frozen Plasma (FFP)
			Units
			Dose
			Infection Risk
			Complications
		Cryoprecipitate
			Content
			Compatibility
			Indications for Cryoprecipitate
			Cryoprecipitate Is Not Indicated for the Following
			Dose
			Complications
		Albumin and Artificial Colloids
			Composition
			Use
			Typical Volume
		References for Further Reading
	Postoperative Hemodynamic Assessment and Management in PCICU
		Background
		Assessment of Cardiac Output
		Inotropic Support
			Catecholamines
		Management of Low Cardiac Output
			Practical Guide
			General Points
		Vasodilators
		Individual Vasodilator Agents
			Milrinone (A Phosphodiesterase Inhibitor)
			Sodium Nitroprusside
			Nitroglycerine (Glyceryl Trinitrate)
			Prostacyclin, Epoprostenol
			Phenoxybenzamine
			Phentolamine
			Captopril
			Labetalol
			Esmolol
			Digoxin
		Volume Replacement
		References for Further Reading
	Sedation, Analgesia and Cerebral Protection After Cardiac Surgery
		Background
		Routine Analgesia/Sedation Orders
		Cerebral Protection After Cardiac Surgery
		References for Further Reading
	Post-cardiac Surgery Seizures
		Background
		Introduction
		Etiology
		Diagnostic Workup
		Supportive Management
		Anticonvulsant Therapy
		References for Further Reading
	Cardiac Arrhythmias in PCICU
		Background
		Assessment
		Narrow Complex Tachycardia
			Sinus Tachycardia
			Nodal Dependent Supraventricular Tachycardia
			Atrial Flutter/Fibrillation
			Junctional Ectopic Tachycardia (JET)
		Wide Complex Tachycardia
			Ventricular Tachycardia
		Bradyarrhythmias
			Physiological
			Pathological
		References for Further Reading
	Capillary Leak Syndrome After Cardiac Surgery
		Background
		Clinical Features
		Who Is at Risk?
		When Does It Present?
		Management
		References for Further Reading
	Fluid, Electrolyte, and Lactate Management After Cardiac Surgery
		Background
		Fluids After Cardiac Surgery
			Day 1 (Day of Surgery)
			Subsequent Days
			Modifications
		Electrolyte Management
			Potassium
			Hypokalemia
			Guidelines for Potassium Supplementation
			Concentrated Potassium Infusions
			Oral Potassium Supplementation
			Hyperkalemia
				Structured Guideline for Management of Hyperkalemia:
					Mild Hyperkalemia (K+ 5.0–6.0)
					Management of Severe Hyperkalemia
			Plasma Ionized Calcium
				Causes of Hypocalcemia in Critically Ill Patients
			Calcium Infusion Regimen (See Also Fig. 1)
				Monitoring
				Precautions
				Calcium Preparations
			Hyponatremia
				Symptoms of Hyponatremia
				Management of Severe Hyponatremia
			Hypernatremia
				Management of Hypernatremia
			Blood Lactate
				Blood Lactate (How It Is Produced and What It Tells Us?)
		References for Further Reading
	Postoperative Renal Function and Acute Kidney Injury
		Background
		What Rate of Urine Output Is Adequate Postoperatively?
		Management of Postoperative Oliguria
		Indications for Dialysis
		Peritoneal Dialysis (PD) Catheter Placement
		Renal Failure During Prolonged Intensive Care
		References for Further Reading
	Infections After Cardiac Surgery in PCICU
		Background
		Elevated Temperature
		Perioperative Prophylactic Antibiotics for Cardiac Surgery
		Line Related Infections
		Prevention of Line Related Infections
		What Does This Mean in Practice?
		VAP (Ventilator Associated Pneumonia)
		CAUTI (Catheter Associated Urinary Tract Infection)
		Surgical Site Infection (SSI)
			Recommended Measures to Help Preventing SSI:
		Necrotizing Enterocolitis (NEC)
		References for Further Reading
	Nutrition and Metabolism in PCICU
		Background
		Nutritional Assessment and Feeding Plan
		Enteral Feeding
			Feeding Methods
			Diarrhea
			Management of Feeding Intolerance
			Composition of Enteral Feeds (See Table 1)
		Parenteral Nutrition
			Components of PN
			Total Parenteral Nutrition
			Complications of Parenteral Nutrition
			Initial Monitoring Required During PN
			Simple PCICU NG Feeding Guidelines
		Minimal Feeding Protocol Flow Chart
		Standard Feeding Protocol Flow Chart
			Blood Glucose Management
			Hypoglycemia
				Causes
				Management
			Hyperglycemia (Glucose >16 mmol/L):
				Causes
				Management
			Glucose Requirements
		Postoperative Glycemia Protocol (PGL) in PCICU
			Metabolic Acidosis
				Common
				Less Common
				Investigations
				Management
		References for Further Reading
	Blalock-Thomas-Taussig (BTT) Shunt: Early and Follow-Up Management
		Background
		Indications for BTT Shunt
		Post-Op BTT Shunt Complications
		Oxygen (O2) Saturation Changes
		Post-operative (OP) Follow-Up Care
			Respiratory System
				Prerequisites for Extubation
			Cardiovascular System
			Coagulation
			Infection
			Fluids/Nutrition
			Management of Post-BTT Shunt Emergencies
		References for Further Reading
	Management of Hypoplastic Left Heart Syndrome in PCICU Before and After Cardiac Surgery
		Background
			Presentation
			Management at Presentation
			Balancing Shunts Preoperatively
			Timing of Surgery
		Surgical Management and Pathophysiology
			Norwood Procedure and Norwood Physiology
			“Norwood Equation” for Pulmonary Shunt Flow Calculation
			Pathophysiological Considerations of Stage 1 Norwood Procedure
		Postoperative Management of Norwood Stage-I
			Balancing Shunts Postoperatively
		QP/QS Flow Calculations
			Shunt Calculation: (SaO2 − SvO2)/(PvO2 − PaO2)
			General Care Postoperatively
		References for Further Reading
	Guideline for Ultrasound Evaluation for Diaphragmatic Dysfunction in Post-operative Cardiac Children
		Background
			Role of Diaphragm as Breathing Muscle in Neonates and Infants
			Indications for US of Diaphragm
			Description of 2D-Mode and M-Mode Ultrasound Technique
			How Can Normal Function of the Diaphragm Determined by US Screening?
			Management
		References for Further Reading
	Pediatric Critical Care Ultrasound: An Essential Skill in Pediatric Cardiac Intensive Care Unit
		Background
		Hemodynamic Assessment
			Gross Evaluation of Right and Left Ventricular Functions
			Vena Cava Analysis
			Assessment of the Lung
		Airway, Hypoxia and Respiratory Assessment
		Neurological Assessment
		Abdominal Ultrasound
		Vascular Access and Other Procedures
		References for Further Reading
	Mechanical Circulatory Support in Pediatric Cardiac Critical Care
		Background
		Indications
		Types of Mechanical Circulatory Support
			Berlin Heart or EXCOR Ventricular Assist Device (VAD)
			Post-operative Care
			Special Safety Considerations
		References for Further Reading
	Notes on Selected Surgical Procedures in CHD and Their Management in PCICU
		Background
		Shunt Lesions with Increased Pulmonary Blood Flow
		Pulmonary Artery (PA) Banding
		Modified Blalock–Thomas–Taussig (BTT) Shunt
			Practical Points
			Management of “Over Shunting” and “Under Shunting” BTT Shunt
			Seroma
		Tetralogy of Fallot (TOF)
			Severe Right Ventricular Dysfunction
		Anomalous Pulmonary Venous Drainage
		Transposition of the Great Arteries (TGA)
			Presentation and Early Management
			Surgery
			Postoperative Care of Patients After Arterial Switch Surgery
		Total Cavo-Pulmonary Connection: TCPC (“Fontan”)
			Postoperative Care
			The “Failing Fontan” Circulation
		Coarctation
		References for Further Reading
	Training in Pediatric Cardiac Intensive Care
		Background
		Traditional Model of Care for Pediatric Critical Patients with Heart Diseases
		Why Do We Need PCICU and Dedicate Trained Team?
		What Are the Components of Specific Medical and Procedural Skills and Training That Are Required for Management of Critical Pediatric Cardiac Patients? See Tables 1 and 2
		Who Can Be Candidate to Be Trained in Management of Pediatric Critical Cardiac Patients?
		Suggested Example of Formal Training Model in Pediatric Cardiac Intensive Care Medicine
		What Are the Center Requirements for Accreditation in Pediatric Cardiac Intensive Care Medicine?
		Pediatric Cardiac Intensive Care Unit for Training Other Disciplines
		Conclusion
		References for Further Reading
Part IV: Clinical Pharmacy Guidelines
	Safe Medication Use in Pediatric Cardiology
		Background
		Sources of Errors in Pediatrics
			Off-Label Use of Medication
		Methods to Improve Medication Safety in Pediatrics
			Clinical Pharmacy Involvement (Unit-Based Pharmacist)
			Technology to Improve Medications Use and Safety
			Practice Guidelines to Improve Safety
		References for Further Reading
	Developmental Pharmacokinetics
		Background
			Common Pharmacokinetic Parameters
		Drug Absorption
		Drug Distribution
		Drug Metabolism
		Drug Excretion
		Pharmacodynamics (PD)
		References for Further Reading
	Precision Medicine: Applications to Pharmacotherapy
		Background
		Components of PM
		Challenges of PM in Pediatrics
		Benefits of PM
		Currents Applications of PM in Pharmacotherapy
		References for Further Reading
	Drug Interactions
		Background
		Types of Drug Interactions
			Drug-Drug Interactions (DDI)
			Drug-Food and Herbal Interactions
			Drug-Disease Interactions
			Drug-Lab Interactions
		References for Further Reading
	Medication Administration via Enteral Feeding (EF) Tube
		Background
			Characteristics of EF Tubes
		Medications Administration via EF tube
			Drug-Feed Interactions
			Contraindications of Medication Administration via EF tube
			Prevention of ET Tube Blockage by Medications
			Tube Flushing for Drug Administration
			Common Medications Administered via EF Tube (Table 1)
		References for Further Reading
	Pharmacotherapy of Apnea
		Background
		Risk Factors of Apnea
		Management of Apnea
		Pharmacotherapy of Apnea
			Caffeine
			Theophylline and Aminophylline
			Doxapram
		References for Further Reading
	Pharmacologic Management of Patent Ductus Arteriosus (PDA)
		Background
		Pharmacologic Maintenance of PDA Patency
			Alprostadil
		Pharmacologic Closure of PDA
			Cyclooxygenase-2 (COX-2) Inhibitors
			Acetaminophen
		References for Further Reading
	Pharmacotherapy of Necrotizing Enterocolitis
		Background
		Risk Factors of NEC
		Modified Bell’s Staging of NEC
		Prevention of NEC
		Pharmacological Therapy
			Feeding Recommendation
			Antimicrobial Treatment (See Table 2)
			Probiotics
		References for Further Reading
	Untitled
	Caring for Patients with Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
		Background
		Classification of G6PD Variants
		General Recommendation for G6PD Patients
			Medications Avoidance in G6PD Patients (Table 2)
		References for Further Reading
	Analgesia and Sedation in Pediatric Cardiac Intensive Care Unit (PCICU)
		Background
		Approach to Sedation and Analgesia in the ICU
			Challenges in Pediatric Cardiac ICU
		Pre-sedation/Analgesia
			Assessment
			Fasting (NPO)
			Staffing and Equipment
			Initiation of Sedation
		Intra-sedation/Analgesia
			Monitoring
			Daily Sedation Interruption (DSI)
			Sedative Agents Cycling
		Post-Sedation/Analgesia
			Recovery
			Criteria for Discharge from Sedation and Analgesia
			Iatrogenic Drug Withdrawal (IDW)
			Management of Withdrawal
		Sedation Protocol
		Pharmacologic Therapy
			Non-opioid Analgesics
			Opioid Analgesics
			Benzodiazepine (BDZ)
			Dexmedetomidine (DMT)
			Clonidine
			Propofol
			Ketamine
			Phenobarbital
			Chloral Hydrate
		Appendix 1. Sedation and Analgesia Protocol
		Appendix 2. Sedative and Analgesic Conversion/Weaning
		Appendix 3. Sedation Assessment Tools
			COMFORT Scale
		Appendix 4. Pain Assessment Tools
			4A: CRIES Scale
			4B: FLACC Scale
			4C: Wong-Baker Faces Scale for
			4D: The Numeric Pain Rating Scale
		Appendix 5. Iatrogenic Drug Withdrawal (IDW) Assessment Tool
			Withdrawal Assessment Tool-1 (WAT-1)
		References for Further Reading
	Pharmacotherapy of Pain
		Background
		Pain Assessment
		General Principles of Pain Management
		Non-pharmacological Management
		Non-opioid Analgesics
		Acetaminophen
		Non-steroidal Anti-inflammatory Drugs (NSAIDs)
		Opioid Analgesics
		Opioid Equivalent Doses and Conversion
		Naloxone for Opioid Reversal (Table 7)
		References for Further Reading
	Delirium in Pediatric Cardiac Intensive Care Unit (PCICU)
		Background
		Features of Delirium
		Risk Factors (Table 1)
		Assessment of Delirium
			Cornell Assessment of Pediatric Delirium (CAPD) (Table 2)
		Approach to the Management of Delirium (Fig. 1)
		Pharmacologic Therapy
		References for Further Reading
	Pharmacotherapy of Seizure
		Background
			Classification of Seizures (Fig. 1)
			Pediatric Comorbidities Associated with Seizure
		Nonpharmacologic Therapy
			Ketogenic Diet
		Pharmacologic Therapy
			Carbamazepine
			Clobazam
			Ethosuximide
			Felbamate
			Gabapentin
			Lacosamide
			Lamotrigine
			Levetiracetam
			Oxcarbazepine
			Phenobarbital
			Phenytoin/Fosphenytoin
			Topiramate
			Valproic Acid
		Status Epilepticus
			Background
			Management of Status Epilepticus: Rapid Sequence Management (Fig. 2)
			Therapy for Status Epileptics (Table 2)
			Therapy for Refractory SE
		References for Further Reading
	Pharmacotherapy of Hypertension
		Background
			Risk Factors of Pediatric HTN
			Classification and Stages of Pediatric HTN
			Clinical Staging of Pediatric HTN
		Hypertensive Crisis: Emergency and Urgency
			Approach to the Management of Pediatric HTN
				First-Line Antihypertensive
			Approach to the Management of the Patient with Elevated BP (Fig. 1)
				Nonpharmacological Management
				Pharmacologic Therapy
					Angiotensin-Converting Enzyme Inhibitors (ACEIs) (Table 7)
					Angiotensin Receptor Blockers (ARBs) (Table 8)
			Calcium Channel Blocker (CCBs) (Table 9)
			β-Blockers (BB) (Tables 10, 11, and 12)
			Thiazide Diuretics (Table 13)
			Sodium Nitroprusside (SNP)
			Hydralazine
			Nitroglycerin
			Phenoxybenzamine
			Phentolamine
			Clonidine
			Minoxidil
			Alpha-Adrenergic Blockers (Prazosin, Terazosin, and Doxazosin) (Table 15)
			Fenoldopam
		References for Further Reading
	Pharmacotherapy of Heart Failure
		Background
		Classification and Stages of Pediatric HF
			The NYHA Functional Classification of HF
			The Ross Modified Functional Classification of Pediatric HF
			The ISHLT Staging Classification of HF
		Acute HF
			Approach to Acute Management
			Diuretics
			Vasodilators, Inotropes, and Vasopressors
			Nesiritide
		Chronic HF
			Approach to Chronic Management
			New Reconsideration
			ACEIs and ARBs
			β-Blockers (BB) (Table 5)
			Sacubitril/Valsartan
			Aldosterone Antagonist: Spironolactone
			Digoxin
			Hydralazine
			Ivabradine
		Other Management Recommendations for Patients with HF
			Anemia and Iron Supplement
			Medications Avoidance in a Patient with HF (Box 2)
			Anticoagulation in HF Pediatric Patients
		References for Further Reading
	Pharmacotherapy of Dyslipidemia
		Background
		Definitions
		Risk Factors of Pediatric DL
			Medications-Induced DL (Table 2)
			DL Associated with Congenital Heart Disease
		DL Screening Recommendation for Pediatrics (Table 3)
		Non-pharmacological Management
		Pharmacologic Therapy
			Statins (Table 4)
			Bile Acid Sequestrants (Resins) (Table 5)
			Ezetimibe
			Nicotinic Acid (Niacin)
			Fibric Acid (Table 6)
			PCSK9 Inhibitors
			Omega-3 Fatty Acids
		References for Further Reading
	Pharmacotherapy of Pediatric Arrhythmias
		Background
		Risk Factors of Arrhythmia
		Common Pediatric Arrhythmias (Table 1)
			Drug-Induced QT: Prolongation
		Management of Pediatric Arrhythmias
			Amiodaron
			Adenosine
			Digoxin
			Procainamide
			Disopyramide
			Lidocaine
			Phenytoin
			Mexiletine
			Flecainide
			Ivabradine
			Propafenone
			Esmolol
			Beta-Blocker (Propranolol, Atenolol, Nadolol)
			Sotalol
			Calcium Channel Blockers (CCB) (Verapamil, Diltiazem) (Table 5)
			Magnesium Sulfate
		References for Further Reading
	Pharmacotherapy of Infective Endocarditis
		Background
		Risk Factors of IE
		Microbiology of Pediatrics IE
		Modified Duke Classification
		Pharmacotherapy of IE
			Empirical Therapy of IE (Table 2)
			Microorganism-Directed Therapy of IE (Table 3)
			Penicillin
			Cephalosporins
			Aminoglycosides
			Vancomycin
			Daptomycin
			Rifampicin
			Antifungals
		Prevention of IE
		References for Further Reading
	Pharmacotherapy of Kawasaki Disease
		Background
		Classifications
			Clinical Phases of KD (Table 1)
			Z-Score Classification of Coronary Artery Abnormalities
		Management of KD
			Approach to Acute Management
			Approach to Chronic Management
		Therapy for KD
			Intravenous Immunoglobulins (IVIG)
			Aspirin
			Steroids
			Infliximab
			Other Options for Treatments of Refractory Cases
		References for Further Reading
	Pharmacotherapy of Myocarditis
		Background
		Triphasic Disease Process of Myocarditis (Table 1)
		Pharmacologic Therapy
			First Phase (Acute Fulminant)
			Second Phase
			Third Phase
		References for Further Reading
	Pharmacotherapy of Pleural Effusion
		Background
		Classification of PE: “Light Criteria”
			Risk Factors of PE
			Common Pathogens Associated with PE
		Non-pharmacological Management
		Pharmacologic Management
			Antibiotic Therapy (Tables 3 and 4)
			Analgesics and Antipyretics
			Thrombolytic Therapy (Table 5)
		References for Further Reading
	Pharmacotherapy of Pericarditis
		Background
		Classification of Pericarditis (Table 1)
		Post-pericardiotomy Syndrome (PPS)
		Pharmacologic Therapy (Table 2)
		References for Further Reading
	Pharmacotherapy of Chylothorax
		Background
		Classifications
		Risk Factors of Chylothorax in Pediatric Cardiac Patients
		Management of Chylothorax (Table 1)
			Octreotide Acetate
			Other Therapy
		References for Further Reading
	Pharmacotherapy Related to VADs
		Background
		Pharmacokinetic Changes Associated with VADs
		Pre-operative Preparation
		Peri-operative Management
			Surgical Antimicrobial Prophylaxis
			Right Ventricle Support
			Prevention of Thromboembolism
		Post-operative Complications
		References for Further Reading
	Immunosuppression Therapy Post Heart Transplant
		Background
		Immunosuppression Therapy (IST): General Principles
		Role of Clinical Pharmacist in Heart Transplant
		Induction Therapy
			Eligibility for Induction Therapy
			Common Immunosuppressants Used for Induction in HTx
		Maintenance Therapy
			Common Immunosuppressants Used for Maintenance in HTx
		Immunosuppressant Therapy
			Steroids
			Anti-Human-T-Lymphocyte Immune Globulin
			Muromonab-CD3 (Orthoclone, OKT3)
			Alemtuzumab
			Daclizumab
			Basiliximab
			Mycophenolate Mofetil (MMF)
			Azathioprine (AZA)
			Tacrolimus (TAC)
			Cyclosporine (CSA)
			Proliferation Signal Inhibitors (PSIs)
		Rejection
			Hyperacute Rejection
			Acute Cellular Rejection (ACR)
			Antibody-Mediated Rejection (AMR)
			Cardiac Allograft Vasculopathy (CAV)
				Suggested Preventive Measures for CAV
				Pharmacotherapy Management of CAV
		Antimicrobial Prophylaxis
			Fungal Infection
			Cytomegalovirus (CMV)
			Pneumocystis Jiroveci Pneumonia (PJP/PCP)
		References for Further Reading
	Anticoagulation
		Background
		Case-Specific Therapeutic Goals and Duration
		Anticoagulants
			Unfractionated Heparin (UFH)
			Enoxaparin (LMWH)
			Warfarin
			Aspirin
			Clopidogrel
			Dipyridamole
			TIROFIBAN for Perioperative Antiplatelet Bridging
			Alteplase (tPA)
			Direct Oral Anticoagulants (DOACs)
		Management of Heparin-Induced Thrombocytopenia (HIT)
		References for Further Reading
	Pharmacotherapy of Perioperative Bleeding
		Background
		Classification of Perioperative Bleeding
			Hemorrhagic Shock Severity Classification (Table 1)
		Risk Factors of Perioperative Bleeding
		Prevention of Postoperative Bleeding
		Non-pharmacological Management
		Pharmacologic Therapy
			Antifibrinolytics
			Vitamin K (Phytonadione)
			Desmopressin
			Recombinant Activated Factors VII (rVIIa)
			Prothrombin Complex Concentrate (PCC)
			Recombinant Activated Factors VIII (Antihemophilic Factor)
			Fibrinogen Concentrates
			Protamine Sulphate
		References for Further Reading
	Inotropes and Vasopressors
		Background
		Definitions
		Facts to Consider
			Receptor Effects and Clinical Actions (Table 1)
		Monitoring of Patient on Inotropes and Vasopressors
			Dopamine
			Dobutamine
			Norepinephrine
			Epinephrine
			Isoproterenol
			Phenylephrine
			Milrinone
			Amrinone
			Vasopressin
			Levosimendan
		References for Further Reading
	Paralytic Agents
		Background
		Selection of Paralytic Agents
		Paralytic Agents
			Depolarizing Agent: Succinylcholine
			Non-depolarizing Agents
		Weaning of Paralysis
			Reversal of Paralysis
		References for Further Reading
	Pharmacotherapy Related to ECMO
		Background
		Pharmacokinetic Changes Associated with ECMO
		Thrombosis Prevention During ECMO
			Unfractionated Heparin (UFH)
			Direct Thrombin Inhibitors (DTIs)
		Medications Management During ECMO
			Cardiovascular Medications (Table 3)
			Analgesics and Sedatives (Table 4)
			Antimicrobials (Table 5)
		References for Further Reading
	Pharmacotherapy of Pulmonary Hypertension
		Background
		Hemodynamic Definitions of PH
			Clinical Classification of PH
		Management of PH
			Phosphodiesterase Type 5 Inhibitors (PDE5i)
			Guanylate Cyclase Stimulator: Riociguat
			Endothelin Receptor Antagonist (ERA)
			Prostacyclin-Targeted Therapy (PG)
			Selexipag
			Calcium Channel Blockers (CCB)
			Inhaled Nitric Oxide (iNO)
			PH Promising Pipeline Therapies
			Other Supportive Therapy
		References for Further Reading
	Postoperative Respiratory Complications
		Background
		Excessive Lung Secretions
		Bronchospasm and Wheezing (Table 2)
			Inhaled Corticosteroid
		References for Further Reading
	Medications Management in Renal Insufficiency
		Background
		Staging of AKI
			KDIGO Staging Criteria
			Pediatric Modified RIFLE (pRIFLE) Criteria
			Pediatric Modified AKIN Criteria
		Assessment of AKI
			Creatinine Clearance Estimation (CrCl)
			Renal Angina Index (RAI)
			Percentage of Fluid Overload (FO)
			Furosemide Stress Test (FST)
			Other AKI Biomarkers: Urine Indices
		Risk Factors of AKI in Pediatric Cardiac Patients
		Prevention of AKI
		Pharmacologic Therapy of AKI
			Fluid Therapy
			Diuretics
			Vasodilators (Table 5)
			Other Options
		Pharmacotherapy of Hydronephrosis (Box 2)
		Medication Adjustment in Renal Insufficiency
			Effects of Dialysis on Drug Clearance
		Medications Adjustment
			Dose Adjustment for Common Cardiovascular Medications (Table 7)
			Dose Adjustment for Common Antimicrobial Agents (Table 8)
		References for Further Reading
	Medication Management in Liver Insufficiency
		Background
		Pathophysiological Changes Affecting Drug Pharmacokinetics
			The Hepatic Extract Ratio
		Assessment of Liver Impairment
			Child-Pugh Classification (Table 1)
			Model for End-Stage Liver Disease (MELD)
		Dosage Adjustment in Liver Dysfunction (Table 2)
			General Strategies
		Drug-Induced Liver Injury (DILI)
			Risk Factors of DILI
			Mechanism of DILI
				Two Major Types of DILI
				Drugs Known to Cause Liver Injury (Table 3)
			Pathological Manifestation of DILI
				Presented in Two Forms
			Suggested Management
		References for Further Reading
	Pharmacotherapy of Gastroesophageal Reflux
		Background
		Non-Pharmacologic Recommendations
		Pharmacologic Management
			Antacid
			Histamine H2 Receptor Antagonists (H2RAs)
			Sucralfate
			Proton-Pump Inhibitors (PPIs)
		References for Further Reading
	Postoperative Nausea and Vomiting
		Background
		Risk Factors of PONV and Assessment
			POVOC Risk Scoring
		Management of PONV
			Preoperative Optimization
			Pharmacologic Prophylaxis
		References for Further Reading
	Pharmacotherapy of Feeding Intolerance
		Background
		Definition of Feeding Intolerance
		Risk Factors of Feeding Intolerance
		Non-pharmacological Management
		Pharmacologic Therapy
			Prokinetics (Table 1)
			Antiemetics (Table 2)
			Acid Suppression Therapy
		References for Further Reading
	Pharmacotherapy of Constipation
		Background
		The Rome III Definitions for Functional Constipation
		Risk Factors
			Medications Known to Cause Constipation (Boxes 1 and 2)
		Non-pharmacological Therapy
		Pharmacological Therapy
			Initial (Disimpaction) and Maintenance Constipation Therapy (Table 1)
			Other Available Therapies (Table 2)
			Newer Therapies (Table 3)
		References for Further Reading
	Stress-Related Mucosal Disease
		Background
		Risk Factors and Prevention of SRMD
			Prevention of Stress Ulcer
		Management of Clinically Significant UGIB
		References for Further Reading
	Glycemic Control in Hospitalized Children
		Background
		Risk Factors of Hyperglycemia During Hospitalization
		Management of Hyperglycemia
			Glycemic Target
			Non-pharmacological Therapy
			Pharmacological Therapy
			Insulin
			Metformin
			Other Diabetes Therapies (Table 5)
				Hypoglycemia (Box 1)
		References for Further Reading
	Pharmacotherapy of Fungal Infections
		Background
		Management of Fungal Infections
			Invasive Fungal Infections (IFIs)
			Superficial Fungal Infections
		Therapy for Fungal Infections
			Polyenes Antifungal (Table 1)
			Azole Antifungal (Table 2)
			Echinocandin Antifungals (Table 3)
			Other Antifungals (Table 4)
		References for Further Reading
	Fluid and Electrolytes Management
		Background
		Fluid Resuscitation
			Types of Intravenous Fluid
		Potassium (K)
			Facts to Consider (Box 1)
		Hypokalemia
			Potassium Replacement (Table 4)
		Hyperkalemia
			Treatment of Hyperkalemia (Table 5)
		Magnesium (Mg)
			Facts to Consider (Box 2)
		Hypomagnesemia
			Magnesium Replacement (Table 6)
		Hypermagnesemia
			Treatment of Hypermagnesemia
		Calcium (Ca)
			Facts to Consider (Box 3)
		Hypocalcemia
			Calcium Replacement (Table 7)
		Hypercalcemia
			Treatment of Hypercalcemia (Table 8)
		Phosphate (P)
			Facts to Consider (Box 4)
		Hypophosphatemia
			Phosphate Replacement (Table 9)
		Hyperphosphatemia
			Treatment of Hyperphosphatemia (Table 10)
		Sodium (Na)
			Facts to Consider (Box 5)
		Hyponatremia
			Sodium Replacement (Table 11)
		Hypernatremia
			Treatment of Hypernatremia
		Sodium Bicarbonate (NaHCO3)
			Facts to Consider (Box 8)
			Bicarbonate Supplement (NaHCO3) (Table 12)
		References for Further Reading
	Parenteral Nutrition
		Background
		Feeding Pre-cardiac Surgery
		Feeding Post Cardiac Surgery
		Parenteral Nutrition (PN)
		Indications of Parenteral Nutrition
		Initial Assessment of Child Nutritional Status (Table 1)
		Peripheral Vs. Central PN (Table 2)
		Total Fluid Requirements
		Nutritional Needs Estimation (Total Energy Expenditure TEE): Tables 5 and 6
		Protein (Amino Acids) Requirements (Table 7)
		Carbohydrates (Dextrose) Requirements (Table 8)
		Lipid (Fatty Acids) Requirements (Table 10)
		Electrolyte Requirements (Table 11)
		Vitamins Requirements (Table 12)
		Trace Elements Requirements
		Other Additives
			Heparin
			H2 Blocker
			Selenium
			Irone Dextran
			Carnitine
			Insulin
		Monitoring of Patients on PN (Table 14)
		PN for Special Population (Table 15)
		PN Complications and Management (Table 16)
		Assessment of Successful PN (Table 17)
		PN Compatibility
		Withholding PN
		Weaning Patient from PN
		Nursing Responsibility
		Cyclic PN
		Home PN
		References for Further Reading
	Nutritional Supplements
		Background
		Children’s Nutritional Needs
		Assessment of Nutritional Risk: STRONGKids Score
		Recommended Nutritional Supplements for Children
			Multivitamins (MV)
			Vitamin D
			Oral Iron Supplement
		References for Further Reading
	Therapeutic Drug Monitoring
		Background
			Definitions
			Indications of TDM
			Drug-Level Interpretation
			TDM of Common Narrow Index Medications
				Vancomycin
				Aminoglycosides
				Other Medications (Table 9)
		References for Further Reading
Part V: Adults with Congenital Heart Disease
	Adults with CHD
		Background
			Tetralogy of Fallot (TOF)
			Special Considerations During Pregnancy and Post TOF Repair
		Transposition of the Great Arteries (TGA)
			Special Considerations During Pregnancy and Post Arterial/Atrial Switch Repair
		Shunt Lesions
			Atrial Septal Defect (ASD) and Anomalous Pulmonary Venous Connection
			Ventricular Septal Defect (VSD)
			Patent Ductus Arteriosus (PDA)
			Special Considerations During Pregnancy and Shunt Lesions
			Eisenmenger Syndrome
		Aortic Stenosis (AS)
			Special Considerations During Pregnancy and Aortic Stenosis
			Aortopathy
			Special Considerations During Pregnancy and Aortopathy
			Coarctation of the Aorta (CoA)
			Special Considerations During Pregnancy and Coarctation of the Aorta
		Ebstein Anomaly
			Special Considerations During Pregnancy and Ebstein Anomaly
			Special Clinical Conditions
		Pulmonary Stenosis (PS)
			Special Considerations During Pregnancy and Pulmonary Stenosis (PS)
		Pulmonary Hypertension (PH)
			Special Considerations During Pregnancy and Pulmonary Hypertension (PH)
		Total Cavopulmonary Connection (TCPC; “Fontan”)
			Special Considerations During Pregnancy and TCPC/Fontan
		References for Further Reading
Part VI: Appendix
	Normal Values/Reference Tables and Graphs in Pediatric Cardiac Care
		Background
		Vital Signs
		Heart Rates at Rest/min
		Normative Blood Pressure Levels by DINAMAPa Monitor in Children Age 5 Years and Younger
		Definition of Hypotension by Systolic Blood Pressure and Age
		DINAMAPa Blood Pressure Percentiles for Neonates to 5-Year-Old Children
		DINAMAPa Blood Pressure Percentiles for Boys 5–17 Years Old (San Antonio Children’s Blood Pressure Study)
		DINAMAPa Blood Pressure Percentiles for Girls 5–17 Years Old (San Antonio Children’s Blood Pressure Study)
			Measurements in Pediatric Cardiac Echocardiography
		M-Mode Measurements of Left Ventricular Dimensions and Wall Thickness
		M-Mode Measurements of the Right Ventricle, Aorta, and Left Atrium
		Two-dimensional Echocardiographic Measurements of the Pulmonary Valve and Pulmonary Arteries
		Two-Dimensional Echocardiographic Measurements of Aortic Root and Aorta
		Two-Dimensional Echocardiographic Measurements of Atrioventricular Valves
		Two-Dimensional Echocardiographic Measurements for Major Coronary Artery Segments
			Pediatric Electrophysiology
		ECG: Q-Wave Voltages According to Lead and Age
			Pediatric Cardiac Catheterization
		McGOON Ratio and NAKATA Index
		AMPLATZER® ASD Occluder
		AMPLATZER® Membranous VSD Occluder
		AMPLATZER® Muscular VSD Occluder
			General Pediatric Cardiology
		Management Algorithm in Pulmonary Atresia with Intact Ventricular Septum (PAIVS)
		Levels of Evidence (LOE) Categories
		Class of Recommendations (COR) Categories