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Absent superior vena cava (SVC) is an asymptomatic congenital systemic venous anomaly which is rarely detected and compatible with normal life. Undiagnosed absent SVC may cause problems during cardiac catheterization or cardiac surgery. We present our surgical experience in a patient with tetralogy of Fallot who had undiagnosed absent SVC.

OBJECTIVES: Neurological complications after cavopulmonary connections like bidirectional Glenn shunt and Fontan connection are occasionally encountered in the postoperative period. We discuss such a case of bilateral bidirectional Glenn shunt which developed convulsive syncope postoperatively. CASE: A 5-year-old cyanotic girl diagnosed as tricuspid atresia with pulmonary stenosis without any spell history underwent bilateral bidirectional Glenn shunt on the way to a subsequent Fontan. After an uneventful surgery she developed convulsive syncope on straining for defecation in the postoperative period. A thorough neurological and arrhythmia study failed to elicit any organic lesions. DISCUSSION: The diagnosis of a neurological event after a single ventricle palliation is paramount to its management. Differentiating syncope from a seizure has its own management implications. The etiologies of neurological complications are varied after cardiac surgery. The physiology and etiology of syncope and seizure after a cavopulmonary connection is discussed. The role of physiological factors in a situation of altered physiodynamics like a bidirectional shunt and Fontan has not been dealt with before in a clinical setting. We have discussed this case to understand the effects of these factors. The effects of strain on the systemic venous pressure, the pulmonary artery pressure and the intrathoracic pressure, can lead to a neurological event if balance is not maintained between the driving pressure of the systemic venous pressure and the pulmonary capacitance. We have devised a simple test to identify these subsets preoperatively by a modification of the Valsalva maneuver. CONCLUSION: Although neurological complications crop up occasionally after single ventricle palliation, not much in-depth analysis has been done regarding the physiological factors involved after such an altered physiology. The effects of systemic venous pressure, the pulmonary artery pressure and the intrathoracic pressure must be in harmony for proper functioning of the shunt; thus strain can alter physiodynamics to such an extent to manifest clinically as a neurological event. The modified Valsalva maneuver can be applied clinically as a 'biomarker' to identify a subset of patients prone for neurological complications.

Anomalous left coronary artery arising from the pulmonary artery is a rare congenital heart disease. Mortality is in excess of 90% if left untreated during the first year of life. Surgical management has evolved from coronary ligation to anatomical repair by coronary button translocation to the aorta. We report our recent experience with surgical correction of this rare condition. We retrospectively studied the case records of 14 consecutive patients operated in our institute between Jan 1998 to Aug 2004. The mean age was 65 months ±114 (Range 1 to 420). The male to female ratio was 2.5:1. Coronary ligation ws performed in two patients, coronary artery bypass grafting with saphenous vein graft was done in three, the last nine patients underwent coronary button transfer, one patient required concomitant mitral valve replacement. Clinical profile, surgical techniques and operative outcomes as well as long-term results were analyzed. Hospital mortality was 28.5% (4/14), Follow up was 100% complete with no late mortality, ranging from 1 months to five years. Ischaemic mitral regurgitation progressed in only one patient, remained the same in one and improved in all the others. All patients except one are in functional class I at last follow up. In conclusion, infants presenting with a diagnosis of anomalous left coronary artery arising from pulmonary artery with congestive cardiac failure are a difficult subgroup of patients. Coronary button translocation has emerged as the single most effective surgical modality and is associated with good long-term results.

A 20 year-old male was diagnosed to have Ebstein's anomaly with severe right ventricular dysfunction. He was taken up for 1.5 ventricle repair. Post procedure, there was difficulty in weaning from cardiopulmonary bypass due to progressive right ventricular dilatation compromising the systemic output. An atrial septectomy did not help. Progressive right ventricular dilatation compressing the left ventricle, demonstrated on transoesophageal echocardiogram, prompted us to perform a right ventricular exclusion and univentricular palliation. The patient was successfully weaned off cardiopulmonary bypass and had a smooth postoperative recovery. Judicious use of right ventricular exclusion and univentricular palliation could be an effective bailout strategy in difficult surgical scenarios in Ebstein's anomaly.

The Warden procedure for the correction of a right-sided partial anomalous pulmonary venous connection to the high superior vena cava is well established. It has the advantages of avoiding sinoatrial node dysfunction and pulmonary and systemic venous obstruction. In the case related here, a 3-year-old girl presented with a superior vena cava type of sinus venosus atrial septal defect and an anomalously draining right upper pulmonary vein, with bilateral superior venae cavae. Our approach to the Warden procedure was through a right posterolateral thoracotomy, which provided additional advantages.

Decisions to operate on patients with shunt lesions presenting late with severe pulmonary arterial hypertension (PAH) and borderline operability are often not based on precise cut off values of haemodynamic data owing to paucity of studies.To assess the reliability of the preoperative haemodynamic data and reactivity test in predicting the postoperative reversibility of PAH in patients with isolated large ventricular septal defects (VSDs) and borderline operability.Between 2004 and 2010, 30 patients underwent VSD closure surgically; no early deaths occurred. Twenty-six patients were followed up regularly (mean 39.6±16 months) and one late postoperative death occurred. Fourteen patients who had been followed up for at least 1 year postoperatively underwent cardiac catheterisation.There were 3 responders (asymptomatic patients with pulmonary vascular resistance (PVR) index3 WU.m(2)) and 12 non-responders. The following were lower among responders: mean age at surgery (3.2±0.42 vs 11.55±3.29 years, p=0.227), mean baseline PVR index (3.69±0.8 vs 10.57±9.1, p=0.204), average resistance ratio (RR=0.25±0.01 vs 0.59±0.25, p=0.049) and ratio of pulmonary and systemic mean pressures (PAm:SAm ratio) (0.70±0.009 vs 0.87±0.118, p=0.003).Preoperative 'base line' PAm:SAm and RR appear to be better predictors of postoperative outcome than other baseline parameters. Preoperative reactivity test had no significant role in predicting postoperative reversibility of PAH at mid-term.

A 25-year-old man in New York Heart Association functional class IV with right ventricular endomyocardial fibrosis received a palliative bidirectional Glenn shunt. Despite a stormy postoperative convalescence the bidirectional Glenn shunt provided good long-term palliation.

A 4-year-old girl with a congenital pulmonary arteriovenous fistula underwent selective ligation of the fistulous tract with the aid of a preoperatively placed endovascular catheter and cardiopulmonary bypass.

A Fontan operation was planned for a 5-year-old girl with complex cyanotic heart disease and bilateral superior venae cavae. The unexpected finding of total anomalous pulmonary venous connection to the left superior vena cava led to a change of strategy. During a bilateral bidirectional Glenn procedure, the pulmonary confluence had to be disconnected to avoid pulmonary venous obstruction. This resulted in bilateral unidirectional cavopulmonary flow.

A 3-year-old female child presented with total anomalous pulmonary venous connection to the coronary sinus coexisting with a persistent left superior vena cava. During the surgical correction, since there was no innominate vein, the left superior vena cava required diversion to avoid systemic desaturation. This was achieved by end-to-side left superior vena cava-to-left pulmonary artery anastomosis that was technically easier than transposing it to the right atrium.

A 30-year-old male with tetralogy of Fallot presented with a history of hemoptysis. He had a major aortopulmonary collateral artery that could not be interrupted during corrective surgery due to technical problems. Postoperative recovery was complicated by congestive heart failure. Embolization of the aortopulmonary collateral artery was planned but he developed massive hemoptysis and emergency transcatheter occlusion was performed, which successfully controlled both hemoptysis and congestive heart failure.

A simplified technique for repair of submitral left ventricular aneurysm is presented. Since the patient had no mitral regurgitation the aneurysm was approached through a left thoracotomy with femorofemoral bypass and the repair was technically simple.

One-and-a-half ventricle repair, consisting of endocardiectomy with tricuspid valve replacement and bidirectional cavopulmonary shunt, was performed on a patient with right ventricular endomyocardial fibrosis and right ventricular outflow tract obstruction. The patient made a smooth recovery. We believe that this repair provides good palliation for a subset of patients with right ventricular endomyocardial fibrosis.

A 33-year-old woman in the postpartum period presented with a mass in the right ventricular outflow tract. She underwent excision of the mass under standard cardiopulmonary bypass. Histopathologic examination of the mass revealed a metastatic lesion from the thyroid, which was follicular carcinoma of the thyroid. Later she underwent total thyroidectomy with lymph node dissection of the neck and radioactive 131I ablation for the residual tumor in the neck. At 1-year follow-up, the patient has no evidence of residual lesion in the heart, neck, or anywhere else in the body. A detailed preoperative workup could have changed the order of interventions and probably avoided a heart operation.

Aortopulmonary collateral arteries (APCA) may be a major source of postoperative morbidi ty in patients with tetralogy of Fallot (TOF) with pu lmonary stenosis or a t res ia . P e r s i s t e n c e of t hese vesse l s in the pos topera t ive per iod may result in hemoptys i s and congestive heart failure. We report a case where oral a n t i c o a g u l a t i o n r e s u l t e d in life t h r e a t e n i n g in t rapulmonary hemorrhage in an adult patient who underwent corrective surgery of tetralogy of Fallot with aortic valve replacement that was managed successfully with percutaneous embolization.

Cor triatriatum is an uncommon but surgically correctable cause of pulmonary venous hypertension and congestive cardiac failure, with a reported incidence of 0.1% among children with congenital heart diseases. Association with partial atrioventricular canal defect (PAVCD) is even rarer, with only anecdotal reports appearing in the literature. In the classic form, cor triatriatum is characterized by the presence of a fibromuscular diaphragm that subdivides the left atrium into a proximal accessory chamber and a distal true chamber.

We report a case of luetic saccular aneurysm of the ascending aorta eroding into the right atrium causing an aorta-to-right atrial fistula. The patient had severe aortic regurgitation, pulmonary arterial hypertension, and congestive cardiac failure. Patch repair of the aneurysm from the aortic side, direct closure of the fistulous opening from the right atrial side, and aortic valve replacement were performed. The patient recovered fully. This case is reported because of its extreme rarity and good surgical result.