The Journey of an Indian Pediatric Cardiologist : Dr. K. C. Chaudhuri Lifetime Achievement Award/Oration at AIIMS, New Delhi, September 2017.

The life journey of an Indian pediatric cardiologist, who bestowed considerable attention to the development of new knowledge and train/teach physicians around the world while providing care of patients with heart disease over a 45-y period, is reviewed. This appraisal focuses particular attention on the scientific contributions to the literature. These include spontaneous closure of physiologically advantageous ventricular septal defects, various issues related to a congenital heart defect namely, tricuspid atresia and transcatheter and, interventional pediatric cardiac procedures.

Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment.

Unilateral absence of pulmonary artery (UAPA) is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. UAPA is often associated with other congenital cardiovascular anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Diagnosis of UAPA is very difficult and is based on taking a complete medical history, physical examination and imaging examinations. Clinical symptoms include exercise intolerance, haemoptysis and recurrent respiratory infections. Adult patients with UAPA are often asymptomatic. There is no consensus regarding the treatment for UAPA. The therapeutic approach should be based on symptoms of the patient, pulmonary artery anatomy and associated aortopulmonary collaterals. Treatment options for these patients include partial or total pneumonectomy, closure of selected collateral arteries not solely responsible for pulmonary blood flow or a primary versus staged pulmonary artery anastomosis. This review summarizes pathophysiology, symptomatology and current diagnosis and treatment of this disease.

Diagnosis and management of cyanotic congenital heart disease: part II.

In this review, the clinical features and management of less commonly encountered cyanotic cardiac lesions are reviewed. Pathophysiology, clinical features, laboratory studies and management are discussed. The clinical and non-invasive laboratory features of these cardiac defects are sufficiently characteristic for the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required. Such studies may be needed either to define features that could not be clearly defined by non-invasive studies or prior to performing trans-catheter interventions. Surgical correction or effective palliation is possible at relatively low risk. But, residual defects, some requiring repeat catheter or surgical intervention, may be seen in a significant percentage of patients and consequently, continued follow-up after surgery is recommended.

Prevalence of undiagnosed congenital cardiac defects in older children.

BACKGROUND: Certain congenital cardiac defects may go undetected for several years due to lack of symptoms and signs. Our purpose was to determine the prevalence of such defects among children greater than one year of age. METHODS: The study was performed on subjects diagnosed with systemic hypertension, aged from 1 to 19 years, with a mean of 12.4 years, in whom we performed echocardiography, using a standard protocol, to establish any end-organ damage or to reveal any congenital cardiac defects. RESULTS: We found a congenital cardiac defect in 5 (3.5%) of the 143 children evaluated. Of these, 4 had not previously been detected, specifically Ebsteins malformation of the tricuspid valve, with moderate regurgitation, a coronary arterial anomaly, a bicuspid aortic valve, and prolapse of the mitral valve permitting regurgitation. In the other patient, we found a non-significant tiny muscular ventricular septal defect. CONCLUSIONS: Our transthoracic echocardiographic investigation revealed previously unsuspected congenital cardiac defects in 4 of 143 older children, with 3 of these requiring further management by a paediatric cardiologist. A similar prevalence has also been reported in older children evaluated echocardiographically for other diseases such as insulin-resistance and leukemia. Hence, it is possible that the prevalence of congenitally malformed hearts is higher than previously reported. When clinically indicated, clinicians should more readily consider obtaining an echocardiogram to help in the identification of such malformations.

Diagnosis and management of acyanotic heart disease: part I -- obstructive lesions.

In this review, the clinical features and management of most commonly encountered acyanotic obstructive cardiac lesions are discussed. Mild lesions, especially in children are usually asymptomatic while neonates and infants may present with symptoms. Ejection systolic murmurs in patients with pulmonic and aortic stenosis and decreased femoral pulses and blood pressure difference (>20 mmHg) between arms and leg in patients with aortic coarctation are usually seen. Clinical diagnosis is not difficult and the diagnosis can be confirmed and quantitated by non-invasive echocardiographic studies. Whereas surgical intervention was used in the past, balloon dilatation appears to be effective in the treatment of these lesions.

Diagnosis and management of acyanotic heart disease: part II -- left-to-right shunt lesions.

In this review, the clinical features and management of most commonly encountered acyanotic, left-to-right shunt lesions are discussed. Patients with small defects, especially in childhood, are usually asymptomatic while moderate to large defects in infancy may present with symptoms. Hyperdynamic precordium, widely split and fixed second heart sound, ejection systolic murmur at the left upper sternal border and a mid-diastolic flow rumble at the left lower sternal border are present in atrial septal defects, holosystolic murmur at the left lower border is characteristic for a ventricular septal defect whereas a continuous murmur at the left upper sternal border is distinctive for patent ductus arteriosus. Clinical diagnosis is not usually difficult and the diagnosis can be confirmed and quantitated by non-invasive echocardiographic studies. Whereas surgical intervention was used in the past, transcatheter methods are increasingly used for closure of atrial septal defect and patent ductus arteriosus. Small ventricular septal defects may not need to be closed whereas medium and large defects may require surgical closure. Transcatheter closure of both muscular and membranous ventricular septal defects is feasible by transcatheter methodology, but these techniques are experimental at the time of this writing.

Successful thrombolytic therapy of pulmonary embolism associated with urosepsis in an infant.

Childhood pulmonary embolism (PE) is a rare but serious condition marked by hypoxemia, shock, right-sided heart failure, and significant risk for fatality. Recommended treatment options include surgical embolectomy, anticoagulation, and thrombolysis. This report describes the successful use of recombinant tissue plasminogen activator to treat PE associated with urosepsis in a 34-day-old infant and reverse severe hemodynamic compromise. Diagnosis of proximal PE and monitoring its treatment were successfully achieved by echocardiography.

Transthoracic Doppler echocardiography of normally originating coronary arteries in children.

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 +/- 23 versus 24 +/- 6 cm/sec, p = 0.0004), increased diastolic flow (16 +/- 15 versus 6 +/- 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% +/- 14% versus 20% +/- 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m2 (r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight (p < 0.001, r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve.

Perinatal circulatory physiology.

CONCLUSION: Fetal circulation is designed to utilize placenta for gas exchange whereas postnatal circulation uses lungs for gas exchange. Fetal circulatory pathways, namely, umbilical vessels, ductus venosus, foramen ovale and ductus arteriosus facilitate placental gas exchange and promote distribution of oxygenated blood to the vital organs of the fetus. Mechanical factors, prostaglandins and low PO2 in the lung keep the fetal circulatory pathways open. Postnatal circulatory changes include elimination of the placenta, development of pulmonary circulation, and closure of fetal circulatory pathways. Postnatal circulatory changes markedly influence the clinical presentation and clinical course of the neonate with congenital heart defects.

Evaluation of cardiac murmurs in children.

CONCLUSION: Cardiac murmur is a common finding. The murmurs are the most common reason for detection of cardiac disease in children. They are divided into systolic, diastolic and continuous types. Careful evaluation of the murmur and other associated findings will often result in making a reasonably correct diagnosis. Occasional use of noninvasive and invasive studies is necessary for accurate diagnosis. However, these studies may often be required for quantitation and prior to surgical intervention.

Syndrome of single ventricle without pulmonary stenosis but with left atrioventricular valve atresia and interatrial obstruction. Palliative management with simultaneous atrial septostomy and pulmonary artery banding.

Case histories of four infants with single ventricle and left atrioventricular (AV) valve atresia, with interatrial obstruction but without pulmonic stenosis, are presented. Relief of interatrial obstruction by surgical or balloon atrial septostomy resulted in a marked fall in the left atrial pressure and the pulmonary artery mean pressure and resistance as well as a marked increase in the left-to-right interatrial shunt, pulmonary blood flow, and systemic arterial oxygen saturation. Because of this predictable fall in pulmonary vascular resistance, concomitant pulmonary artery banding was performed in each case with good results. There are only a few previously reported cases of single ventricle with left AV valve atresia. Interatrial obstruction is quite common in this lesion. The concept that relief of interatrial obstruction produces a rapid and predictable fall in the pulmonary vascular resistance and that concomitant pulmonary artery banding is necessary in these infants with complex congenital heart disease is emphasized.