pediatrics
Overview of Congenital Cardiovascular Anomalies
Treatment of Congenital Heart Disease
Medical stabilization of heart failure (eg, with diuretics, angiotensin-converting enzyme [ACE] inhibitors, beta-blockers,digoxin,spironolactone, salt restriction and, in selected cases, supplemental oxygen or prostaglandin E1) Surgical repair or transcatheter intervention Treatment of heart failure varies widely depending on the etiology. Definitive therapy typically requires correction of the underlying problem. After medical stabilization of acute heart failure symptoms or cyanosis, most children require surgical or transcatheter repair; the exceptions are certain ventricular septal defects that are likely to become smaller or close with time or mild valve dysfunction. Transcatheter procedures include Balloon atrial septostomy for palliation of severely cyanotic neonates with Transposition of the Great Arteries (TGA) None Balloon dilation of severe Aortic Stenosis or Transcatheter closure of cardiac shunts (most often Atrial Septal Defect (ASD) and Transcatheter placement of pulmonary valve Balloon dilation with or without stenting of vascular stenoses, most commonly pulmonary artery stenosis Acute, severe heart failure or cyanosis during the first week of life is a medical emergency. Secure vascular access should be established, preferably via an umbilical venous catheter. When critical congenital heart disease is suspected or confirmed, an IV infusion of prostaglandin E1 should be started at 0.05 to 0.1 mcg/kg/minute. Keeping the ductus open is important because most cardiac lesions manifesting at this age are ductal-dependent for either systemic blood flow (eg, Hypoplastic Left Heart Syndrome , critical Mechanical ventilation is often necessary in critically ill neonates. Supplemental oxygen should be given judiciously or even withheld because supplemental oxygen can decrease pulmonary vascular resistance, which is harmful to infants with certain defects (eg, hypoplastic left heart syndrome). Other therapies for neonatal heart failure include diuretics, inotropic drugs, and drugs to reduce afterload. The diureticfurosemideis given as an initial bolus of 1 mg/kg IV and titrated based on urine output. Infusions of the inotropesdopamineordobutaminecan support blood pressure but have the disadvantage of increasing heart rate and afterload, thus increasing myocardial oxygen consumption. They are infrequently used in infants with congenital heart disease.Milrinone, frequently used in postoperative patients with congenital heart disease, is both a positive inotrope and a vasodilator.Dopamine,dobutamine, andmilrinoneall have the potential to increase the risk of arrhythmias.Nitroprusside, a pure vasodilator, may be used for postoperative hypertension. It is started at 0.3 to 0.5 mcg/kg/minute and titrated to desired effect (usual maintenance dose is about 3 mcg/kg/minute). and sodium-glucose cotransporter-2 (SGLT-2) inhibitors, may be useful, but data are limited in the pediatric population ( Treatment references ). has been shown to reduce mortality in single-ventricle patients after the Norwood procedure and before the second stage surgery ( Treatment references ). Use of Supplemental oxygen may lessen hypoxemia and alleviate respiratory distress in heart failure; when possible, fractional inspired oxygen (FIO2) should be kept<40% to minimize the risk of pulmonary epithelial damage. Supplemental oxygen must be used with caution, if at all, in patients with left-to-right shunt lesions or left heart obstructive disease because it may exacerbate pulmonary overcirculation. In general, a healthy diet, including salt restriction, is recommended, although dietary modifications may be needed depending on the specific disorder and manifestations. Heart failure increases metabolic demands and the associated dyspnea makes feeding more difficult. In infants with critical congenital heart disease, particularly those with left heart obstructive lesions, feedings may be withheld to minimize the risk of Necrotizing Enterocolitis . In infants with heart failure due to left-to-right-shunt lesions, enhanced caloric content feedings are recommended; these feedings increase calories supplied and do so with less risk of volume overload. Some children require tube feedings to maintain growth. If these measures do not result in weight gain, surgical repair of the anomaly is indicated. Guidelines of the American Heart Association for Prevention ( Unrepaired cyanotic congenital heart disease (including children with palliative shunts and conduits) Completely repaired congenital heart disease during the first 6 months after surgery if prosthetic material or a device was used Repaired congenital heart disease with residual defects at or adjacent to the site of a prosthetic patch or prosthetic device Mechanical or bioprosthetic valve Previous episode of endocarditis 1.Loss KL, Shaddy RE, Kantor PF: Recent and Upcoming Drug Therapies for Pediatric Heart Failure.Front Pediatr9:681224, 2021. Published 2021 Nov 11. doi:10.3389/fped.2021.681224 2.Oster ME, Kelleman M, McCracken C, et al: Association ofdigoxinwith interstage mortality: Results from the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use Dataset.J Am Heart Assoc5(1): e002566., 2016. 3.Bolin EH, Lang SM, Tang X, et al:Propranololversusdigoxinin the neonate for supraventricular tachycardia (from the Pediatric Health Information System).Am J Cardiol119(10): 1605–1610, 2017. 4.Baltimore RS, Gewitz M, Baddour LM, et al: Infective Endocarditis in Childhood: 2015 Update: A Scientific Statement From the American Heart Association.Circulation132(15):1487–1515, 2015. doi:10.1161/CIR.0000000000000298 