Anatomically corrected malposition: report of six cases.
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Biomedical subjects
Publications and source records attributed to B Dalvi.
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Fibrosis is an ubiquitous process. Any tissue injury can culminate in fibroblast accumulation and multiplication with collagen synthesis and deposition. A large number of chronic disease states such as rheumatic heart disease, constrictive pericarditis, cirrhosis of the liver, renal interstitial fibrosis, chronic interstitial lung disease are characterized by extensive fibrosis. In many of these patients, when there is no clinical or laboratory evidence of previous injury, it is presumed that the initiating insult/injury had been 'subclinical'. I propose that 1) the fibroblasts can be activated even in the absence of preceding inflammation, 2) it is the type of 'fibroblast clone' in a given individual together with the 'milieu' in a particular tissue/organ which decides the occurrence and severity of subsequent fibrosis. This fibroblast clonal theory adds a new dimension to the pathogenesis of various disease states and may help in identifying those at high-risk and in evolving a unified therapeutic strategy for amelioration of various disorders characterized by extensive fibrosis.
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Interruption of the aortic arch is a rare and usually lethal cardiac malformation. We report a rare case of a patient with IAA distal to the left subclavian artery associated with double outlet right ventricle, subaortic VSD and patent DA who survived to adulthood. In this patient, the complete diagnosis was made by cardiac catheterization and angiocardiography. We discuss the probable mechanisms, diagnostic problems and therapeutic implications of such long survival.
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Obstructive total anomalous pulmonary venous drainage (TAPVD) has a florid presentation in the neonatal period from the resulting pulmonary edema. A complete anatomical and functional diagnosis is usually possible by transthoracic color Doppler echocardiography, and cardiac catheterization with its attendant risks can usually be avoided in the sick neonate. Currently balloon atrial septostomy has a limited role in palliation of these neonates. Corrective surgery on urgent basis has gratifying results and prevents progression of pulmonary vascular occlusive disease. Pulmonary hypertensive crisis in the early post-operative course needs to be avoided and treated vigorously if it were to develop. Late post-operative course can be complicated by anastomotic obstruction or progressive narrowing of the individual pulmonary veins. Although the former can be dealt successfully by re-operation, surgical treatment of the latter is not satisfactory. Balloon dilatation of the obstructed pulmonary venous pathways, native and post-operative, has been reported with equivocal results. Infant heart-lung transplant is a viable option in patients with diffusely narrow pulmonary veins or complex TAPVDs.
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Twenty children (mean age 3.25 years) with congenital cyanotic heart disease undergoing modified left Blalock-Taussig (BT) shunt were studied. The mean follow-up period was 9.5 months (range 6 months to 1 year). The shunt was performed for cyanotic spells in 15 (75%) and hypoplastic pulmonary arteries in 5 (25%) patients. There were no immediate or late complications. None had cyanotic spell after the shunt. The mean arterial oxygen saturation improved from 66.47 +/- 11.9 to 76.97 +/- 8.16% (p = 0.0003) and mean hematocrit decreased from 51.55 +/- 9.5 to 46.5 +/- 9.7 (p = 0.002) after the shunt. The left atrial systolic volume and left ventricular diastolic volume also increased significantly following the shunt (from 15.82 +/- 6.37 to 20.83 +/- 8.91 ml p = 0.006 and from 36.13 +/- 16.08 to 41.08 +/- 20.07 ml (p = 0.01) respectively. There was significant growth of main, right and left pulmonary arteries and pulmonary valve annulus after the procedure.
Superior vena cava obstruction following corrective repair of total anomalous pulmonary venous return has rarely been described in the literature. A one-month-old boy who underwent corrective surgery for obstructive supracardiac total anomalous pulmonary venous return with consequent symptomatic superior vena cava obstruction in the immediate postoperative period, is reported. This was treated by balloon dilatation followed by stenting of the superior vena cava. The immediate postoperative result was satisfactory and the infant continued to remain asymptomatic at six months follow up. We suggest that this intervention could prove to be a viable alternative to a repeat surgical procedure for such complex cases.
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Patients with pericardial constriction show a prominent 'y' descent in right atrial and vena caval pressure traces. In all earlier hemodynamic descriptions of constrictive pericarditis, the 'y' descent has been described as 'brisk', 'sharp' or 'rapid' but no effort has been made to quantify the same. In this study, we have tried to objectively evaluate and describe this 'y' descent by measuring its negative slope (-dy/dt) at its steepest portion. Forty one patients were studied hemodynamically, 9 with constrictive pericarditis (Group I) and 32 normals (Group II). The negative slope of the 'y' descent in patients with constrictive pericarditis (69.95 +/- 23.04 mm Hg) was found to be significantly greater than normals (35.13 +/- 7.84 mm Hg, p < 10(-6). Discriminant analysis was used to determine its sensitivity, specificity, predictive value and overall accuracy, in the diagnosis of pericardial constriction. Value of > or = 45 mm Hg/sec was found to have the highest overall accuracy (0.88). The correlation between the right ventricular end diastolic pressure and the slope of 'y' descent in patients with pericardial constriction (r = 0.66) and in normals (r = 0.60) was fair. It is concluded that -dy/dt is significantly different in patients with constrictive pericarditis as compared to normals. The diagnostic utility of this parameter needs to be evaluated in patients with equivocal clinical and hemodynamic data, in those with occult pericardial constriction and in post-pericardiectomy cases where the pressures do not normalise immediately after adequate pericardial resection.