Abstract
In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs require intervention, mostly by surgery. Different types of tetralogy of Fallot require different types of total surgical corrective procedures, and some may require initial palliation, mainly by modified Blalock–Taussig shunts. Babies with transposition of the great arteries with an intact ventricular septum as well as those with ventricular septal defects (VSD) need an arterial switch (Jatene) procedure while those with both VSD and pulmonary stenosis should be addressed by Rastelli procedure. These procedures may need to be preceded by prostaglandin infusion and/or balloon atrial septostomy in some babies. Infants with tricuspid atresia require initial palliation either with a modified Blalock–Taussig shunt or banding of the pulmonary artery and subsequent staged Fontan (bidirectional Glenn and fenestrated Fontan with extra-cardiac conduit). Neonates with total anomalous pulmonary venous connection are managed by anastomosis of the common pulmonary vein with the left atrium either electively in non-obstructed types or as an emergency procedure in the obstructed types. Babies with truncus arteriosus are treated by surgical closure of VSD along with right ventricle to pulmonary artery conduit. The other defects, namely, hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, double-outlet right ventricle, double-inlet left ventricle and univentricular hearts largely require multistage surgical correction. The currently existing medical, trans-catheter and surgical techniques to manage cyanotic CHD are safe and effective and can be performed at a relatively low risk.
Highlights
In the first paper [1], management of acyanotic congenital heart defects (CHDs) was discussed.In this paper, discussion of most common cyanotic CHDs will be included
After the diagnosis is confirmed by echo-Doppler and/or cardiac catheterization studies, as needed, a more permanent way to provide pulmonary blood flow should be instituted since the effectiveness of keeping the ductus open with prostaglandin E1 (PGE1) decreases with increasing age
In neonates with markedly decreased pulmonary blood flow with low O2 saturations, the ductus should be made to open by intravenous infusion of PGE1 in a manner similar to that described in Tetralogy of Fallot (TOF) and TGA sections
Summary
In the first paper [1], management of acyanotic congenital heart defects (CHDs) was discussed. Cyanotic CHDs usually have multiple defects of the heart that result in right-to-left shunt. The chronic arterial hypoxemia increases red blood cell production by stimulating erythropoietin from the kidneys If this is excessive, polycythemia will result. Long-standing hypoxemia and polycythemia result in coagulation abnormalities and hyperuricemia These complications may be prevented by maintaining an optimal level of hemoglobin, avoiding dehydration, and by performing timely palliative or corrective surgery. If not all cyanotic CHDs need intervention, the format of this paper will be different from that used for acyanotic CHDs. For each of the cyanotic CHD, a brief description of the defect complex will be included followed by timing and methods of management concurrently; palliative and corrective therapies are discussed as appropriate for a given lesion. Exercise restriction is not necessary unless symptoms develop with activity
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