Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication

ustification: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However,these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required whenrecommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and mayhave co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National ConsensusMeeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of MedicalSciences, New Delhi. The meeting was supported by Children’s HeartLink, a non-governmental organization based in Minnesota, USA.Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heartdiseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenitalheart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductusarteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heartdiseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebsteinanomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Prevalence of rheumatic heart disease: Has it declined in India.

Rheumatic heart disease continues to be a major health problem in many parts of the world. The epidemiology of rheumatic heart disease in India is of special interest as it may help to understand the effects of economic transition on this enigmatic disease. Critical appraisal of the published literature suggests the possibility of a real decline in the occurrence of the disease in some parts of the country, but a continuing onslaught in several other regions. The rate of decline seems to correlate more with improved public health facilities than with economic development alone. However, the cumulative burden of the disease remains high, and sustained efforts for the prevention of rheumatic heart disease are warranted.

Secundum ASD--not for closure.

A 32-year-old man with 17 mm secundum atrial septal defect showed hemodynamic deterioration during temporary balloon occlusion of the defect. The closure was not done. Further investigation led to the diagnosis of primary cardiac amyloidosis.

Congestive heart failure in unoperated tetralogy of Fallot: can hypoxia be a cause?

A 10-year-old boy with tetralogy of Fallot and congestive heart failure underwent a right-sided modified Blalock-Taussig anastomosis because of severe biventricular dysfunction and repeated hypercyanotic spells. Postoperatively, there was improvement in systemic oxygen saturation and myocardial function. We postulate that congestive heart failure occurred because of severe myocardial hypoxia and its elimination resulted in markedly improved cardiac performance.

Surgical outcome of staged univentricular-type repairs for patients with univentricular physiology and pulmonary hypertension.

BACKGROUND: There is no consensus about the most appropriate limits of pulmonary artery pressure and vascular resistance in case of patients undergoing univentricular or one and one-half ventricular repair. This study was conducted to analyze the mortality and morbidity of a heterogenous group of patients with a functionally univentricular heart and pulmonary artery hypertension, undergoing pulmonary artery banding followed by univentricular-type repairs. METHODS AND RESULTS: Out of 254 patients undergoing pulmonary artery banding for a functionally univentricular heart with increased pulmonary blood flow, 148 patients underwent definitive second stage surgery. Post-band hemodynamic evaluation revealed persistently high pulmonary artery pressure (> 18 mmHg), and pulmonary vascular resistance (>2.0 Woods units/m2) in 78.3% patients. Sixteen patients with moderate right ventricular hypoplasia were given a one and one-half ventricle repair (Group I), 82 patients a bidirectional Glenn connection (Group II), and 50 patients a fenestrated total cavopulmonary connection (Group III). The overall mortality following second stage surgery for the high pulmonary artery pressure group (n=116) was 30.17%, while none of the low pulmonary artery pressure group died (p=0.0009). Pulmonary hypertensive crises and/or systemic desaturation were the main causes of death at second stage repair. All mortality occurred in patients with mean pulmonary artery pressure > 18 mmHg and pulmonary vascular resistance > 3.5 Woods units/m2. Survivors from this group had persistent morbidity in the form of superior vena caval syndrome and suboptimal oxygen saturation (70-75%). CONCLUSIONS: It is advisable not to proceed with definitive second stage repair if post-pulmonary artery banding mean pulmonary artery pressure is over 25 mmHg and pulmonary vascular resistance exceeds 4.0 Woods units/m2. These patients may possibly be deemed to have undergone definitive palliation during their pulmonary artery banding.

Radiofrequency perforation for pulmonary atresia and intact ventricular septum.

Surgical or interventional perforation of atretic pulmonary valve as initial palliative procedure has been advocated in patients with pulmonary atresia with an intact ventricular septum. We report two infants with pulmonary atresia and intact ventricular septum, who were treated by radiofrequency perforation and balloon dilatation.

Dilated cardiomyopathy in Indian children.

BACKGROUND: The aim of this study was to ascertain the clinical course and prognosis of dilated cardiomyopathy in Indian children. METHODS AND RESULTS: The records of 82 children with dilated cardiomyopathy (50 males), less than 12 years of age (mean age 2.9+/-3.07 years), were retrospectively reviewed. Clinical variables, laboratory parameters, and serial echocardiograms were analyzed. On a mean follow-up of 25.09 months (range 15 days-118 months), 9 out of 78 patients died (11.5%) (CI: 4.5%-18.5%). Mortality was 25% (6/24) in infants but the actuarial survival was 87% at 5 years in those diagnosed beyond infancy. Serial echocardiograms of 66 patients (80%) were available. Of these, 39 patients (59%) (CI: 47%-70%) improved, 12 (18%) (CI: 9%-27%) deteriorated or died, and 15 (23%) (CI: 13%-33%) remained unchanged during the follow-up. Among the prognostic variables, only age less than 1 year, higher cardiothoracic ratio, and a higher ratio of left ventricular diastolic dimension/posterior wall thickness was associated with a poor outcome on univariate analysis. CONCLUSIONS: Dilated cardiomyopathy in children pursues a heterogeneous course with a high mortality in infants. A large number of children diagnosed beyond infancy improve or recover. Further characterization of prognostic variables is warranted.

An infant with "dying spells".

A 45-day-old infant presented with the unusual and intriguing symptom of episodic crying and loss of consciousness. The infant was discovered to have a vascular compression of the trachea by the innominate artery, almost serendipitously. He was cured of his symptoms by anterior suspension of the innominate artery.

Isolation of the left subclavian artery.

Two cases of isolation of the left subclavian artery from the aortic arch are reported for the rarity of this lesion. One patient was diagnosed clinically, the other after angiography. The isolated left subclavian artery was reimplanted in one patient. This rare anomaly has clinical and surgical relevance and should be diagnosed by diligent clinical and angiographic evaluation.

Medium-term outcome of anatomically repaired congenitally corrected transposition: the double switch operation.

BACKGROUND: The double switch operation is emerging as the procedure of choice for congenitally corrected transposition of the great arteries. However, rhythm disturbances in the postoperative period are rarely discussed. METHODS AND RESULTS: Eighteen survivors who underwent corrective surgery for congenitally corrected transposition of the great arteries were followed up. Patients in group I (n=8), who also had a ventricular septal defect and pulmonary stenosis, had undergone the Senning plus Rastelli operation. Patients in group II (n=10), who did not have pulmonary stenosis, had undergone the Senning and arterial switch operation. The patients were followed up by periodical clinical examination, echocardiography and 24-hour Holter monitoring. In group I, follow-up ranged from 24 to 66 months (mean 44 months). There was no late death and all the patients are symptom free. There was no significant atrioventricular valve regurgitation and left ventricular function was normal. There were no rhythm disturbances. In group II, follow-up ranged from 2 to 72 months (mean 48 months). There were 2 late deaths due to atrial tachyarrhythmia and residual pulmonary hypertension 36 and 8 months after the procedure, respectively. One patient had significant mitral regurgitation and required mitral valve replacement. Three patients had recurrent atrial/junctional tachyarrhythmia: one of them was lost to follow-up after 1 year while another died of resistant atrial tachyarrhythmia. The third patient underwent mitral valve replacement for severe mitral regurgitation and developed complete heart block necessitating a permanent pacemaker implantation. CONCLUSIONS: Though good long-term results are obtained following the double switch operation, the problem of atrial arrhythmias still needs to be addressed suitably.

Chronic oral sildenafil therapy in severe pulmonary artery hypertension.

BACKGROUND: Sildenafil, a selective phosphor-diesterase-5 inhibitor, may be of clincal benefit in patients with pulmonary artery hypertension. METHODS AND RESULTS: Fourteen patients, aged 5-30 years, with severe pulmonary artery hypertension (9 with primary pulmonary hypertension, 5 with operated congenital heart disease) received oral sildenafil in addition to conventional therapy. Twelve patients were in New York Heart Association functional class III or IV. The drug was started in low dose and empirically increased. Finally a median dose of 87.5 mg/day was used in children weighing less than 30 kg, and 150 mg/day in those with weight more than 30 kg. The patients were followed up by assessing their functional status, six-minute walk test, Doppler echocardiography and hemodynamic study (in selected cases). On mean follow-up of 7.3+/-2.4 months (range 3-14 months), New York Heart Association functional class improved from 3.31+/-0.75 to 2.00+/-0.71 (p<0.002). There was a remarkable improvement on the six-minute walk test from a baseline of 264.1+/-193.7 m to 408.2+/-156.97 m at 3 months (p<0.001) and 453.2+/-159.81 (p<0.0001) at 6 months. The right ventricular systolic pressure estimated echocardiographically declined from 112.40+/-45.21 mmHg to 101.86+/-47.86 mmHg (p<0.002). The mean pulmonary artery pressure decreased from 62 mmHg to 47 mmHg in 4 patients of primary pulmonary hypertension recatheterized after a mean of 7 months of sildenafil treatment. Clinical improvement was seen even when no decrease in pulmonary artery pressure was demonstrated in one patient with secondary pulmonary artery hypertension. However, 2 patients died during follow-up despite clinical improvement. CONCLUSIONS: Oral sildenafil was well tolerated and led to an improved clinical condition and exercise performance. Whether the drug improves mortality remains to be established. Larger trials a rewarranted.