Raghavannair Suresh Kumar

Right ventricular diastolic function after repair of tetralogy of Fallot.

The objective of this study was quantitate diastolic dysfunction in the postoperative phase of tetralogy of Fallot (TOF) and to correlate it with the type of surgical procedure and clinical parameters. Fifty consecutive patients (mean age, 5.0 years; mean weight, 13.5 kg), operated for TOF during the period November 2004 to May 2005, were prospectively studied [infundibular resection, 23; infundibular resection and transannular patch (TAP), 19; right ventricle→pulmonary artery conduit, 8). Detailed echocardiography was done on postoperative days 3 and 9 with a focus on Doppler indices of right ventricular (RV) function, Antegrade late diastolic flow in the right ventricular outflow tract (RVOT) was taken as the marker of restrictive RV physiology. The previous parameters were correlated to the type of surgery and clinical indices of RV dysfunction. There was no mortality. Twenty-four patients showed restrictive RV physiology. This finding correlated with lower values of E/A ratio (0.98 ± 0.17 vs 1.33 ± 0.49, p < 0.002), tricuspid valve E-wave deceleration time (86.9 ± 21.7 vs 151.4 ± 152 msec, p < 0.05), index of myocardial performance (0.15 ± 0.06 vs 0.26 ± 0.09, p < 0.001), isovolumic relaxation time (19.4 ± 17 vs 39±30 msec, p < 0.009), and a higher central venous pressure (15.1 ± 1.5 vs 12.7 ± 1.9, p < 0.001). Restrictive RV physiology correlated with prolonged intensive case unit (ICU) stay (5.1 ± 3.7 vs 2.8 ± 2 days, p < 0.015), longer duration of inotropic support (108.3 ± 56.2 vs 55.5 ± 28.3 hours, p < 0.02), and higher dosage of diuretics. RV diastolic dysfunction is demonstrable by Doppler echocardiography in the first week following surgery for TOF and tends to be worse with TAP. Restrictive physiology demonstrated by RVOT pulse Doppler predicts longer duration of inotropic support, prolonged ICU stay, and higher dosage of diuretics.

Surgical outcome of congenital heart disease in Down’s syndrome

Background and objective: only a few studies have compared surgical outcomes in children with and without Down’s syndrome. The purpose of this study was to investigate the surgical outcome in children with Down’s syndrome and congenital heart disease and to compare it with that in age- and sex-matched non-Down controls. Patients and methods: the charts of 32 consecutive Down’s syndrome patients who underwent surgery for congenital heart disease in a single center from January 2006 to May 2010, were compared with the data of 32 age- and sex-matched non-Down controls, focusing on comparative echocardiographic details, surgical outcome, and intensive care unit data. The data were compared using the 2-group t test. The children were followed up for a median period of 48 months. Results: the most common cardiac lesion in the study group was ventricular septal defect (41%) followed by atrioventricular septal defect (28%). Ventilation and intensive care unit stay were significantly longer in Down’s syndrome children, with a trend towards more respiratory complications. At a median follow-up of 48 months, there were no cardiac events or mortality in the study group. Simple lesions were more common than atrioventricular septal defect in Down’s syndrome. Conclusion: children with Down’s syndrome can be operated on with negligible mortality and good functional outcome, but with a higher surgical morbidity.

Pulmonary artery banding for univentricular heart beyond the neonatal period

Background It is standard practice to band the pulmonary artery at 2 to 4 weeks of age in patients with univentricular hearts with increased pulmonary blood flow. The behavior of patients banded beyond the neonatal period has not been well elucidated. Patients and methods This was a retrospective chart review of 32 consecutive patients (one neonate) who underwent pulmonary artery banding for functionally univentricular heart. The mean age at banding was 5.7 ± 6.0 months, and 34.4% were over 6-months old. Results Mortality was 15.6%. The mean systolic pulmonary artery pressure decreased from 43.6 ± 9.7 to 29.6 ± 7.0 mm Hg. The mean pre-discharge echocardiographic band gradient was 60.6 ± 13.6 mm Hg (mean systemic systolic pressure 73.7 ± 11.0 mm Hg) and systemic oxygen saturation was 81.7% ± 5.8%. At a mean follow-up period of 44.9 ± 30.0 months, 6 patients were lost to follow-up, 13 had undergone bidirectional Glenn shunt, and 7 had Fontan operations. Pulmonary artery mean pressure was 17.2 ± 4.6 mm Hg at pre-Glenn catheterization. Of the 5 patients who had not undergone further surgery, only one was inoperable. All were in functional class I or II. Conclusion Pulmonary artery banding beyond the neonatal period in suitable patients with univentricular hearts provides reasonable palliation in the intermediate term, with a significant number successfully undergoing Fontan stages.

Visceral Heterotaxy in the Developing World

BACKGROUND The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail. METHOD Retrospective chart review of 69 consecutive patients over ten years assessed the clinical profile and surgical outcome of VH. Median age: 3 years; median weight: 15kg. Diagnosis was made by echocardiography supplemented with blood smear (Howell Jolly bodies), Multi-Detector Computed Tomography (MDCT) angiogram and/or surgical inspection. RESULTS In right isomerism (RI) group (n=32), 12 patients did not undergo surgery, five had Blalock Taussig shunt, 14 had bidirectional Glenn and one had Fontan completion, with surgical mortality of 5%. In left isomerism (LI) group (n=31), 11 patients underwent two ventricle repair (35%) and 15 (48%) had single ventricle repair, with surgical mortality of 3.8%; five did not have surgery. On follow up (median period 1.5 years), 33% of un-operated patients and 25% of operated patients died, mortality being higher for RI patients. Late mortality was due to sepsis, heart failure or arrhythmia. CONCLUSION VH can be diagnosed by imaging based criteria. VH tends to present late in the developing world with a significant percentage inoperable. LI had better surgical outcome and higher long term survival.

Scimitar Syndrome with Right Hemianomalous Pulmonary Venous Drainage into Superior Vena Cava/Right Atrium Junction

Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.

Occult systemic lupus erythematosus with active lupus nephritis presenting as Libman-Sacks endocarditis

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. A previously healthy 14-year-old girl presented with Libman-Sacks endocarditis involving the aortic valve as the first manifestation of SLE. Even though she did not satisfy the American College of Rheumatology criteria for diagnosing SLE, she had anemia, proteinuria, elevated erythrocyte sedimentation rate, low complement 4 (C4) levels, and strongly positive antinuclear antibody titer. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with immunosuppressants and steroids. This type of presentation may be misdiagnosed as infective endocarditis missing the underlying collagen vascular disease.

Prosthetic reconstruction of bicuspid pulmonary valve in tetralogy of Fallot.

Background Various techniques have been described for preserving pulmonary valve function in tetralogy of Fallot repair. In selected substrates, the pulmonary valve can be bicuspidized and preserved using polytetrafluoroethylene pericardial membrane. Methods This study was a retrospective review of 20 patients aged 11 months to 31 years with tetralogy of Fallot and a bicuspid pulmonary valve (with anteroposterior cusps) who underwent intracardiac repair from August 2010 to January 2013. The anterior cusp was augmented using polytetrafluoroethylene pericardial membrane to preserve the valve hinge. A transannular patch was used in all cases. Data relating to surgical outcome, intensive care unit course, pulmonary regurgitation, and right ventricular outflow tract gradient were collected. Results There was no mortality. Predischarge pulmonary regurgitation was ≤grade 2 in 18 (90%) patients. One patient had an outflow gradient >40 mm Hg. On follow-up of 3–24 months, there was no increase in outflow gradient; 18.7% had progression of pulmonary regurgitation. Conclusion In selected substrates, this technique is associated with minimization of pulmonary regurgitation and an excellent functional outcome. The utility of this technique needs to be validated over a longer time scale in a larger series.

Absent pulmonary valve syndrome coexisting with coarctation of aorta in an adult.

The combination of absent pulmonary valve syndrome with coarctation of aorta is extremely uncommon and pathogenetically intriguing. This unusual association, which defies popular theories of pathogenesis of coarctation, may need to be looked at in the light of the current concepts regarding the morphogenesis of ventricular outflow tracts and aortic arch and the role of neural crest cells in both. A 19-year-old man with this combination underwent single-stage surgical correction though median sternotomy. The surgical management of this condition in adulthood has not been reported before.

Phenotypic Variations in the 22q11 Deletion Syndrome - Study in a South Indian Population.

To the Editor : The 22q11 deletion syndrome, involving microdeletions in the human chromosome 22q11 region, are associated with multiple phenotypic outcomes [1, 2]. We report identification of these deletions in three cases among a sample of 55 South Indian subjects with congenital heart defects. The analyses carried out using two molecular techniques, RT-PCR and Multiplex Ligation Probe Amplification (MLPA) (MRC HolandKit no. p250 lot# 0509) [3, 4], and further confirmed using cytogenetic techniques of karyotyping and fluorescent in situ hybridization (FISH) provide evidence for the effectiveness of simpler, cost effective and rapid diagnoses.

Factor VII for excessive bleeding following congenital heart disease surgery

Recombinant activated factor VII may be effective in patients with severe bleeding following congenital heart disease surgery requiring cardiopulmonary bypass. From August 2009 through June 2011, 13 patients (median age, 5 months) were given recombinant activated factor VII for severe bleeding after open heart surgery, preventing sternal closure 2 h after completion of cardiopulmonary bypass, or chest tube drainage >10 mLċ kg−1ċh−1 for 2 h. The median dose was 75 µgċ kg−1. The response was positive if postoperative bleeding decreased, permitting sternal closure in the operating room, or if there was > 50% decrease in chest tube drainage. The 3 patients who received factor VIIa in the operating room had successful sternal closure within 1 h. Of the 10 patients who received factor VIIa in the intensive care unit, drainage decreased to < 50% in 1 h in 6 cases, and a second dose was required in 4. None required surgical reexploration. There were 4 deaths, none was related to bleeding. Nine patients were discharged (median hospital stay, 29 days) and were doing well after 3 months. There were no thrombotic complications. Recombinant activated factor VII may be an effective rescue therapy for severe postoperative hemorrhage.