Tachycardiac storm in infant with WPW syndrome: "rescue" radiofrequency ablation.

A two-month-old child having WPW syndrome and orthodromic tachycardia was on treatment with digoxin, flecainide and amiodarone. Despite this, he continued to have severe, very frequent episodes of tachycardia. The left-sided accessory pathway was hence ablated via a patent foramen ovale.

Treatable cardiomyopathies.

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of "treatable cardiomyopathy" include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.

Treatable cardiomyopathies.

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of "treatable cardiomyopathy" include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.

Intrapulmonary vascular dilatations with hypoxemia in extrahepatic portal vein obstruction.

Several mechanisms have been suggested for the development of hypoxemia in cirrhosis. A few patients of portal hypertension due to non cirrhotic liver disease with cyanosis have also been reported earlier. We report probably the first documented case of portal hypertension with portal cavernoma and a normal liver, who had intrapulmonary vascular dilatations leading to hypoxemia and cyanosis. Our case suggests that changes leading to hypoxemia can occur due to portal hypertension alone, in the presence of a normal liver.

Balloon angioplasty for native aortic coarctation.

From May 1987 to August 1990, eighteen patients underwent balloon angioplasty for native aortic coarctation. The age of the patients ranged from four to fifty six years (mean age 17.5 years). The procedure was successful in all cases with a reduction in the peak gradient across the coarctation from 61 +/- 19 mm Hg to 11.7 +/- 8.1 mmHg (p < 0.05). The coarcted segment increased from 4.5 +/- 1.9 mm to 10.7 +/- 3.9 mm (p < 0.05). Peak gradient at six to twelve months follow up, obtained in ten patients, was 19.8 +/- 10.1 mmHg (p = NS). There were no life threatening complications, although seven patients had local vascular problems after the procedure. In two patients, there was persistence of hypertension necessitating drug therapy. On haemodynamic and angiographic restudy in 10 patients, one patient had restenosis and none had aneurysm formation. We conclude that balloon angioplasty is a safe, and less invasive alternative to surgery for native aortic coarctation with gratifying immediate and short term results.

Long term results of percutaneous transluminal valvuloplasty in patients with valvular aortic stenosis.

The results of percutaneous balloon aortic valvuloplasty (PBAV) in 62 consecutive patients with valvular aortic stenosis are reported. The age of the patients ranged from 11 months to 72 years (mean 28 +/- 12 years). Hemodynamically successful dilatation was achieved in 58 out of 62 patients. This was associated with marked clinical improvement in these patients. The left ventricular aortic peak to peak gradient decreased from 96.67 +/- 38.4 to 28.14 +/- 26.5mmHg (p < 0.01). There were no deaths during the procedure. Only one patient died in the hospital during the same admission. There was an increase in aortic regurgitation (AR) by at least one grade in 25 (40.3%) patients. Femoral arterial thrombosis was seen in 9/62 patients, 5 of them requiring surgical intervention. Follow up was available in 28 (45.1%) patients over a period of 2-15 months (mean 9 +/- 3 months). Two patients died during the follow up period. Doppler evaluation of gradients was done in all 28 patients with 15 consenting to undergo repeat cardiac catheterisation. Although hemodynamically the restenosis rate was 35.7% (10/28), only 2 of these patients showed symptomatic deterioration. The success of dilatation and restenosis rate were independent of the etiology of aortic stenosis, presence of calcification and the number of balloons used. This study demonstrates that PBAV is feasible in valvular aortic stenosis at low risk and is able to produce significant clinical and hemodynamic improvement in most cases with a restenosis rate of 35.7% at a follow up period of 9 +/- 3 months.

Congenital intermittent atrio-ventricular dissociation in tetralogy of Fallot (a case report).

A case of tetralogy of fallot with congenital intermittent atrioventricular dissociation is reported. A review of standard postgraduate books of cardiology fails to describe this condition, thus showing its extreme rarity. The therapeutic dilemma in treating such a case is also discussed.

Mortality determinants and prediction of outcome in high risk newborns.

The aim of this study was to determine independent patient-related predictors of mortality in high risk newborns admitted at our centre. The study population comprised 100 consecutive newborns each, from the premature unit (PU) and sick baby care unit (SBCU), respectively. Thirteen high risk factors (variables) for each of the two units, were entered into a multivariate regression analysis. Variables with independent predictive value for poor outcome (i.e., death) in PU were, weight less than 1 kg, hyaline membrane disease, neurologic problems, and intravenous therapy. High risk factors in SBCU included, blood gas abnormality, bleeding phenomena, recurrent convulsions, apnea, and congenital anomalies. Identification of these factors guided us in defining priority areas for improvement in our system of neonatal care. Also, based on these variables a simple predictive score for outcome was constructed. The prediction equation and the score were cross-validated by applying them to a 'test-set' of 100 newborns each for PU and SBCU. Results showed a comparable sensitivity, specificity and error rate.

Cardiac amyloidosis--a case report.

A 75-year-old male patient presented with resistant congestive cardiac failure. Echocardiographic examination revealed normal left ventricular size with symmetrical hypertrophy and generalised hypokinesia. In addition, there was a characteristic granular sparkling appearance of the myocardium. Cardiac catheterization showed biventricular diastolic restriction with normal coronary arteries. Possibility of cardiac amyloid was considered, and the diagnosis was confirmed on rectal biopsy.