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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.

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 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.

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.