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Biomedical subjects

Shyam S Kothari

Publications and source records attributed to Shyam S Kothari.

At least 19 recordsLinked to original sources
PubMed2024

Severe pulmonary arterial hypertension in congenital sideroblastic anemia from PUS1 mutation - a case report.

BACKGROUND: Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate. Severe pulmonary arterial hypertension has not been previously reported in patients with PUS1 gene mutation. CASE REPORT: A 17 year old girl with congenital sideroblastic anemia presented with worsening of breathlessness. Severe pulmonary artery hypertension was documented on investigations. A homozygous variant in exon 3 of gene PUS1,( chromosome 12:g.131932301 C > T c.430 C > T) was found on sanger sequencing. CONCLUSION: We document severe pulmonary arterial hypertension in a patient of congenital sideroblastic anemia from PUS1 gene. We hypothesis that cross talk with TGFb pathways might occur in PUS1 mutation, and that might cause severe PAH. This observation might have therapeutic implications.

Humans↗
PubMed2006

Intra-aortic balloon counterpulsation in a patient with the failing Fontan circulation.

We report a 23-year-old patient undergoing functionally univentricular repair who developed cardiac failure due primarily to systemic ventricular dysfunction. The failing Fontan circulation was successfully re-established using intra-aortic balloon counterpulsation. Aortic counterpulsation facilitates recovery of systemic ventricular function, and appears to be a reasonable alternative in select instances of cardiac failure in patients with the Fontan circulation.

Adult↗
PubMed2006

Criterions for selection of patients for, and results of, a new technique for construction of the modified Blalock-Taussig shunt.

BACKGROUND: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients. PATIENTS AND METHODS: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluoroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation. RESULTS: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months. CONCLUSIONS: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those with disproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.

Anastomosis, Surgical↗
PubMed2006

Sewing needle in the heart.

An unusual case of a sewing needle in the heart is reported for its rarity. The relevant literature is briefly reviewed.

Cardiac Surgical Procedures↗
PubMed2005

Intermediate-term results of percutaneous transvenous mitral commissurotomy in children less than 12 years of age.

The objectives of this study were to review the efficacy of percutaneous transvenous mitral commissurotomy (PTMC) in young children less than 12 years of age and to provide intermediate-term follow-up data. There is a paucity of information regarding the long-term efficacy of PTMC done in children less than 12 years of age. The data of 100 consecutive children less than 12 years of age (mean, 11.1 +/- 1.2 years) who underwent PTMC using Inoue balloon were analyzed retrospectively. Serial clinical and echocardiographic follow-up information of more than 6 months was available in 94 patients. The procedure was successful in 94 patients. The mean calculated mitral valve area (MVA) increased from 0.72 0.14 to 1.7 0.35 cm(2) (P 0.0001). Echocardiographic restenosis (MVA < or = 1 cm(2) or > 50% gain loss) occurred in 14 of 94 patients (16%) over a mean follow-up of 34.4 25.9 (range, 2-115) months. The improvement in New York Heart Association (NYHA) functional class was maintained in most patients (from a mean of 2.87 0.5 pre-PTMC to 1.42 0.6 at follow-up). Seven out of 14 patients with restenosis underwent a re-PTMC. The actuarial rate of good functional status (survival, no repeat interventions, and NYHA class 1 or 2) at 100 months was 75.4% 8.7%. PTMC provides excellent intermediate-term palliation even in young children with rheumatic mitral stenosis.

Age Factors↗
PubMed2005

Right pulmonary artery to left atrium communication.

This is a collective review of 59 published cases of right pulmonary artery to left atrium communication in the English-language literature. In this article, we review the literature on the clinical presentation, anatomic variations, diagnostic features, and management considerations of previously reported cases. Two-dimensional echocardiography and cardiac catheterization including selective angiography provided the necessary diagnostic information and defined the anatomy before surgery in all patients. Various management options including medical, surgical, and transcatheter closure have been reported. The lesions have been classified into four types (I, II, III, and IV) that have a bearing on the appropriate surgical approach and techniques of repair that are discussed in the text. Use of extracorporeal circulation may be considered in selected instances. The operative mortality rate during the earlier era (1950 to 1979) was high, at 22%. More recently, the mortality rate has approached zero. An individualized surgical approach depending on the type of surgical connections is recommended.

Adolescent↗
PubMed2004

Postoperative assessment of the univentricular repair by dynamic radionuclide studies.

BACKGROUND: The purpose of this investigation was to determine the role of radionuclide studies in evaluating postoperative Fontan hemodynamics and to quantify its diagnostic accuracy. METHODS: One hundred five patients (105), aged 11 months to 35 years old, who had undergone univentricular repair, underwent first-pass and multigated acquisition scan 1 month to 10 years after univentricular repair. Forty-five patients with evidence of Fontan failure underwent radionuclide studies using Technetium-99 m as well as cardiac catheterization (group 1). The remaining sixty randomly selected patients with excellent functional status received radionuclide studies alone (group 2). The receiver operating characteristic curve analysis was done to quantify the diagnostic accuracy of the first-pass study. RESULTS: There was paradoxical filling of the right lung after femoral injection in all cases of tunnel or conduit obstruction. A first-pass transit time of 16 to 25 seconds (mean +/- standard deviation [SD] = 18.82 +/- 2.69) was always associated with Fontan failure and high right atrial pressure (range = 20 to 24 mm Hg, mean +/- SD = 22.02 +/- 1.58). A first-pass transit time of 16 seconds was associated with a sensitivity of 100% and a specificity of 93.33%. The predictive accuracy of a positive or negative result was 91.8% and 100% respectively. The area measured under the receiver operating characteristic curve indicates that 99.41% (SE +/- 0.0035) of the time, the value of first-pass time is higher for the Fontan failure group (group 1) compared to the normal group (group 2; p = 0.000). CONCLUSIONS: Our data indicate that Fontan circuit can be reliably evaluated for both anatomic and functional flaws by radionuclide studies; radionuclide first-pass time may be used to predict the chances of Fontan failure postoperatively as well as its presence; and in the presence of atrial fibrillation with fast ventricular rate, analysis using first-pass radionuclide may be impossible and gated equilibrium radionuclide angiocardiography may be the preferred method. Inspection of the systemic ventricular time-activity curve is of crucial importance in this regard.

Adolescent↗
PubMed2004

Histopathology and morphometry of radial artery conduits: basic study and clinical application.

BACKGROUND: The purpose of this study was to determine the pathohistology, morphometry, and risk factors for the development of intimal hyperplasia, calcification, and arteriosclerosis in the radial artery and to compare the morphometry of the distal and proximal radial arteries. METHODS: A total of 190 proximal and distal radial artery specimens obtained from patients who underwent myocardial revascularization were exposed to histopathologic and morphometric analysis. The severity of disease was evaluated on the basis of the percentage of luminal narrowing, intimal thickness index, and intima-to-media ratio. RESULTS: Sixty-two proximal (32.6%) and 22 distal (11.5%) radial artery segments were indicated as histologically normal. Morphometric analysis (Z test) revealed a lesser degree of intimal hyperplasia and luminal narrowing in the proximal segments compared with the distal segments (p < 0.001). The incidence of intimal hyperplasia, medial calcification, and arteriosclerosis in the distal radial arteries was 76.3%, 6.3%, and 5.78%, respectively. Using multivariate logistic regression, we have identified three significant predictors for intimal hyperplasia. Expressed as an odds ratio with a 95% confidence interval, these included (i) age greater than 50 years (1.052; 1.000-1.106, p = 0.052), (ii) smoking (14.073; 5.293-37.414, p = 0.000), and (iii) hypertension (2.777; 1.171-6.583, p = 0.020). Factors associated with an increased likelihood of medial calcification and arteriosclerosis included a history of smoking, diabetes, hypercholesterolemia, peripheral arterial disease, and chronic renal failure (p < 0.05). CONCLUSIONS: The great majority of radial artery conduits indicate preexisting intimal hyperplasia mostly affecting the distal portion. Therefore in cases of longer diseased segments of radial arteries, the discarded segments should be the distal end. Care should be taken when selecting radial artery as a conduit in myocardial revascularization, particularly in elderly males, diabetics, smokers, hypertensive patients, and in those with associated peripheral vascular disease.

Adult↗
PubMed2004

Viable extracardiac total right heart bypass: technical considerations and mid-term results.

BACKGROUND: Direct connection of both caval veins to the pulmonary arterial system would be the ideal right heart bypass by virtue of elimination of prosthetic material and extensive atrial suture lines. However, it can be applied only to selected patients with peculiar anatomical arrangements. We decided to perform total extracardiac right heart bypass using pedicled in situ pericardium in situations when a direct connection was not possible. METHODS: A cohort of five patients, aged 3.5, 4, 10, 10 and 31 years, respectively, with complex cyanotic congenital heart diseases were candidated to extracardiac total cavopulmonary connection using viable in situ pericardium. Aortic cross-clamp was completely avoided in all patients. RESULTS: There was no early or late death. Postoperatively, all patients are in functional class I and in normal sinus rhythm at a mean follow-up of 30 months (S.D.=+/-19 months). Postoperative serial echocardiograms revealed wide, unrestricted anastomoses, unobstructed venous flow to both pulmonary arteries with cardiac pulsations and respiratory variations in the pulmonary arteries due to compressible lateral tunnel. Cavopulmonary angiograms (n=5) in late postoperative period have demonstrated a smooth, nonturbulent, somewhat pulsatile flow without pressure gradient. CONCLUSIONS: The technical advantages and haemodynamic benefits of this operation are encouraging. A longer follow-up is needed to confirm the early results, especially late atrial dysrrhythmias, pulmonary venous obstruction, thromboembolic complications and growth of the conduit.

Journal Article↗
PubMed2002

Alternative approach for angioplasty of stenosed left pulmonary artery following intracardiac repair of tetralogy of Fallot.

A 5-year-old boy with stenosed left pulmonary artery following total correction of tetralogy of Fallot underwent surgical pulmonary arterioplasty through a left anterolateral thoracotomy on a normothermic perfused heart under cardiopulmonary bypass. We found this to be a convenient approach, and recommend it for correction of this lesion in the absence of gross pulmonary regurgitation and right ventricular outflow tract dilatation.

Journal Article↗
PubMedDate not supplied

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.

Adolescent↗
PubMedDate not supplied

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.

Administration, Oral↗
PubMedDate not supplied

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.

Angiography↗