Sitaraman Radhakrishnan

Intermediate and long-term followup of percutaneous device closure of fossa ovalis atrial septal defect by the Amplatzer septal occluder in a cohort of 529 patients

Objectives: The aim of present study is to analyze the intermediate and long-term follow up results of percutaneous closure of fossa ovalis atrial septal defect (ASD) with Amplatzer septal occluder (ASO) in a large cohort of patients including children and adults. Methods: Between May 1998 and July 2008, 529 patients (age group 2-77 years, median 28 years) underwent successful device closure with an ASO at single tertiary referral cardiac center in India.. This was out of an attempted 543 cases. The procedure was carried out in catheterization laboratory under transesophageal echocardiographic and fluoroscopy guidance. The mean size of ASD was 20 mm (7-40 mm) while size of septal occluder was 10-40 mm (mean 24 mm). Two devices were deployed in four patients. Three patients developed transitory pulmonary edema in immediate postprocedure period requiring ICU care for 48 hrs. All patients were advised for Aspirin (3-5 mg/kg, maximum 150 mg) once daily for 6 months. In patients with device 30 mm or larger, Clopidogril ( 75 mg once daily) was given for 3 months in addition to Aspirin. Clinical evaluation, echocardiogram were done on 3 months, 6 months and then at 1, 3, 5, 7 and 10 years of follow up. Transesophageal echocardiography (TEE) was performed in case of any doubt on clinical evaluation or on transthoracic echocardiography (n=10). Results: Followup data is available for 496 patients (93.7%). Followup period is from 12 months to 120 months (median 56 months). On followup, device was in position in all patients, no residual shunt and no evidence of thrombosis. Interventricular septal motion normalized on day of procedure in 89% patients, in 6% over 3 months while flat septal motion persisted in 5% (n=25, all in age group > 40 years) of cases, though right ventricular dilatation persisted in 10% (n=50, age more than 40 years) of patients. Symptom-free survival was 96.7 % (480/496) in patients who came for followup. Only one 68 year old patient with preexistent tricuspid regurgitation developed congestive heart failure, and one patient (58 years old) had a history of hemiparesis after 1 year of device on telephonic interview. Ten patients were in atrial fibrillation (AF) before the procedure and remained in AF on followup. Conclusions: Our study showed that percutaneous closure of fossa ovalis ASD is a safe and effective procedure on intermediate and long-term followup in both the children as well as adults.both. Technical factors during the procedure and proper follow up are important. Our single centre intermediate and long term experience in a large number of patients support the use of device closure as an alternative to surgery.

Hypocalcemic cardiomyopathy presenting as cardiogenic shock

Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated.

Natural history and predictors of moderate mitral regurgitation following balloon mitral valvuloplasty using Inoue balloon

BACKGROUND We studied the predictors and natural history of moderate mitral regurgitation following valvuloplasty using Inoue balloon since it has not been well documented in a large series. METHODS Balloon mitral valvuloplasty was performed in 590 consecutive patients with severe mitral stenosis with mitral regurgitation of mild or lesser grade. Echocardiography and haemodynamics of patients who developed moderate mitral regurgitation were compared with those who did not. They were followed-up. Factors that predicted the development of moderate regurgitation were studied. RESULTS 21 patients (3.5%) developed moderate regurgitation (identified by auscultation, haemodynamics, angiography and colour flow mapping). They were managed conservatively. At 3 months, regurgitation decreased in severity to mild grade in 12 patients. At 1 year, it was trivial in 5, mild in 11 and remained moderate in 5. There was progressive symptomatic improvement. No clinical, echocardiographic, hemodynamic or procedural variables could predict the development of moderate mitral regurgitation. CONCLUSIONS Patients with moderate regurgitation after mitral valvuloplasty show gradual improvement in regurgitation and symptoms. There were no factors-clinical, echocardiographic, hemodynamic or procedural-that predicted the occurrence of moderate MR after BMV.

Left atrial dilatation in constrictive pericarditis: A pre and post-operative echocardiographic study

Thirty-three surgically proven cases of constrictive pericarditis were studied pre-operatively by echocardiography for left atrial dilatation and 18 of them underwent post-operative study at a mean follow-up period of 229 +/- 105 days. The degree of left atrial dilatation in these patients was compared with patients of restrictive cardiomyopathy (n = 8) and sex matched controls of similar age (n = 33). Significant left atrial dilatation was present in patients of constrictive pericarditis compared to controls and it was of a similar or greater degree compared to patients of restrictive cardiomyopathy (the left atrium to aorta ratio was 1.7 +/- 0.31, 1.53 +/- 0.18 and 1.07 +/- 0.1, in constrictive pericarditis, restrictive cardiomyopathy and controls, respectively, P = NS for constrictive pericarditis vs. restrictive cardiomyopathy and P < 0.001 for constrictive pericarditis vs. controls). There was no correlation of degree of left atrial dilatation with clinical and hemodynamic variables. Postoperatively, there was regression of left atrial size in patients with normal hemodynamics (n = 12, pre- vs. post-operative left atrium to aorta ratio 1.65 +/- 0.23 vs. 1.32 +/- 0.14, P < or = 0.001) and persisting or increasing left atrial dilatation in patients with persisting hemodynamic abnormality (n = 6, left atrium to aorta ratio 1.66 +/- 0.23 vs. 1.82 +/- 0.15, P = NS). We conclude that significant left atrial dilatation is a consistent echocardiographic feature of constrictive pericarditis. Hemodynamic normalization following successful pericardiectomy is associated with regression of atrial size.

Echocardiographic demonstration of resolving intrapericardial mass in tuberculous pericardial effusion

A patient with tuberculous pericardial effusion is described who presented with cardiac tamponade. Subsequent to pericardiocentesis, a large echodense intrapericardial mass was observed which disappeared completely with antituberculous chemotherapy. The resolution of the mass suggests that it was a conglomeration of fibrinous exudates deposited in the pericardial cavity. Presence of such exudates should not be considered an indication for surgical intervention.

Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated

We present three unusual cases of total anomalous systemic venous drainage (TASVD) where all the systemic veins were abnormally connected to the atrium that drained into the left ventricle (LV). All three patients had features consistent with left atrial isomerism. Based on the available evidence and review of literature we propose that TASVD be included as a part of the syndrome of left atrial isomerism. A classification for TASVD is also proposed with surgical implications.

Hypoplastic right ventricle with mild pulmonary stenosis in an adult

A rare cyanotic heart disease surviving to adulthood with minimal symptoms is presented. The final diagnosis was hypoplastic right ventricle with mild pulmonary stenosis, where the latter was not responsible for the right-to-left shunting across a large atrial septal defect. The differences from cases reported in the literature are highlighted.

Rosai-Dorfman Disease of Right Atrium Mimicking Myxoma

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare multisystemic disorder that was first reported by Rosai and Dorfman in 1969. It is a distinct histioproliferative disorder due to overproduction of histiocytes, which accumulate in lymph nodes. The cardiac involvement of this disease is extremely rare, and until now, only 18 cases have been reported. We report the case of a 53-year-old woman with right atrial mass mimicking myxoma, which the histopathologic evaluation revealed to be Rosai-Dorfman disease of the right atrium.

Fetal left ventricular myocardial performance index: Defining normal values for Indian population and a review of literature

Objective: The aim of this study was to determine normal values for fetal left ventricular (LV)-myocardial performance index (MPI) in Indian population and to assess its relation to advancing gestation and fetal heart rate (FHR). Materials and Methods: Two hundred pregnant women without any pregnancy-related complications and whose fetuses were shown to have structurally normal hearts were enrolled in this study. Doppler waveform involving simultaneous display of mitral inflow and LV outflow was obtained in all. Various intervals including isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT), and ejection time (ET) were measured and then the MPI was calculated using the formula IVCT + IVRT/ET. Also the correlation between MPI and gestation age and FHR was assessed. We also reviewed the literature on the use of MPI for the assessment of fetal LV function. Results: The normal MPI in second and third trimester fetuses of Indian population was 0.42 ± 0.03. The mean IVCT was 33 ± 4 milliseconds (ms), mean IVRT was 39 ± 5 ms, and mean ET was 169 ± 9 ms. The mean heart rate was 148 ± 8 bpm and the mean PR interval was 111 ± 10 ms. There was no significant association of LV-MPI with either FHR or advancing gestation. Conclusion: MPI is a useful parameter for the assessment global cardiac function. MPI has the advantage of not being affected by FHR, ventricular size, and geometry or image quality. The review of literature shows its significant importance in monitoring complicated pregnancies.

Inferior-type caval vein defect--echocardiographic and surgical description of a large series of patients.

AIM This study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings. METHODS The records of 19 patients - 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography - were retrospectively reviewed and compared with surgical findings. RESULTS Surgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect - confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous. CONCLUSIONS Echocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.