There are several potential issues that affect the treatment and diagnostic pattern of anomalous left coronary artery arising from the pulmonary artery. We report three cases of infants who presented with anomalous left coronary artery arising from the pulmonary artery with severe left ventricular dysfunction and severe mitral regurgitation along with associated anomalies. One patient had congenital lobar emphysema of the right midde lobe. Another patient had left main stem bronchus compression, collapse of basal segments of left lower lobe and panlobular emphysema in medial basal segment of right lower lobe. The third patient had cleft lip and palate. All patients underwent successful repair. The hemodynamic stability was compromised when the infant with congenital lobar emphysema had spontaneous pneumothorax after extubation and she needed an intercostal drainage. The infant with lung collapse had to be reintubated on the second day since she became hypoxic due to recollapse of the lung once the airway positive pressure was removed. She needed chest physiotherapy, vigorous endotracheal suctioning and inhaled bronchodilator therapy. The patient who had cleft palate succumbed to aspiration pneumonitis in the postoperative period. Follow-up of other two patients after three months showed very good improvement in left ventricular systolic function.

Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.

Total anomalous pulmonary venous connection becomes a totally different subset when associated with complex congenital anomalies. The combination of two separate life-threatening congenital heart defects complicates the management of these patients. Six patients with total anomalous pulmonary venous connection associated with complex congenital heart disease were studied. There were 2 girls and 4 boys. Three of them were less than 5 kg in weight, and the other 3 were more than 5 kg in weight. Four patients had severe pulmonary arterial hypertension and 2 patients had pulmonary stenosis. Three patients had supracardiac type with a right vertical vein, one had drainage to the right atrium superior vena cava junction, one patient had supra cardiac type but split flow to both the superior vena cava and one patient had cardiac type. Three patients had double outlet right ventricle. Three patients had atrioventricular canal defect and 2 patients had preoperative pulmonary vein obstruction. All patients underwent rerouting of pulmonary veins. Concomitant procedures included intraventricular tunnel repair of ventricular septal defect and infundibular resection in double outlet right ventricle. Atrioventricular canal repair was done for Rastelli type A atrioventricular canal. Superior vena caval plasty, atrioventricular canal repair and pulmonary artery banding were done in unbalanced atrioventricular septal defect and large double outlet right ventricle. Intracardiac repair through transatrial approach was done for tetralogy of Fallot. Right ventricle-pulmonary artery conduit was done for truncus arteriosus. Single ventricle repair was done for corrected transposition of great arteries. There were 2 hospital deaths.