Surgical management of aortopulmonary septal defect.

Fourteen patients with malseptation of the aortopulmonary trunk underwent operative repair from 1978 to 1988. Age ranged from 1 year to 35 years (mean age, 9.6 years). Five patients had type I, 6 had type II, and 3 had type III lesions. The hemodynamic disturbance in all patients was the consequence of a large left-to-right shunt (mean pulmonary/systemic flow ratio, 2.38:1) with increased pulmonary vascular resistance (mean value, 4.47 units/m2). Our initial surgical experience with closure under cardiopulmonary bypass through the transaortic route in 3 patients and the transpulmonary approach in two patients resulted in 3 deaths. In the 9 subsequent patients, division and repair of the defect in the great vessels yielded uniformly good results. During follow-up, which ranged from 3 months to 2 years, all 11 survivors had good clinical improvement and none showed residual defects on restudy. Pulmonary artery pressure and pulmonary vascular resistance decreased in all patients except 1.

Pulmonary wedge aspiration cytology in the diagnosis of recurrent tumour embolism causing pulmonary arterial hypertension.

Recurrent subacute pulmonary embolism leading to pulmonary arterial hypertension is an uncommon presentation of malignancy. We describe one such patient who presented to us with features of pre-capillary pulmonary arterial hypertension. A novel yet simple technique, involving pulmonary wedge aspiration cytology, provided the tissue diagnosis of trophoblastic malignancy, thus enabling prompt institution of chemotherapy and consequent impressive clinical improvement. This technique appears to be a hitherto unreported indication for intravascular catheterisation in the diagnostic work up of pulmonary arterial hypertension.

Clinical profile and natural history of Ebstein's anomaly of tricuspid valve.

There were 63 patients of Ebstein's anomaly of tricuspid valve encountered from 1976 to 1991; 28 (44.4%) were male and 35 (55.6%) female. Their age at presentation ranged from 3 months to 51 years. Five (7.9%) patients were asymptomatic, 48 (76.2%) had class II-III exertional dyspnoea, palpitation or both. Thirty patients (47.6%) had cyanosis. Electrocardiogram showed paroxysmal atrial fibrillation in two, chronic atrial fibrillation in four (6.3%), paroxysmal supraventricular tachycardia in seven, atrial or ventricular ectopic beats in five (7.9%), 2:1 atrioventricular block in one (1.6%), complete atrioventricular block in two (3.2%) and type B WPW syndrome in nine patients (14.3%). Chest X-ray showed diminished vascularity in 22 (34.9%). Diagnosis was established by cardiac catheterization and or echocardiography. Atrialized right ventricular chamber was demonstrated in 51 (80.9%) by angiography and in 40 (63.5%) by electrophysiology. Patients were followed up for 1-172 months. Seventeen patients (26.9%) required surgery. Three patients (4.8%) died during medical follow-up, and five (7.9%) died following surgery. Survival probability for 46 medical patients was 88.9% at 172 months. Factors affecting survival were pulmonary blood flow, cyanosis, clubbing and systemic arterial oxygen saturation.

Subvalvar aneurysms of the left ventricle.

Five cases of subvalvar aneurysm of the left ventricle are reported. The location of the aneurysm was sub-mitral in three and sub-aortic in two. The clinical, echocardiographic, angiocardiographic and pathological features are described.

Prognosis for patients with Eisenmenger syndrome of various aetiology.

The objective of this study was to determine the long-term survival pattern and variables affecting long-term survival and complications occurring during follow-up of patients with Eisenmenger syndrome. A retrospective study of patients diagnosed with Eisenmenger syndrome were followed up. A tertiary care centre was used and it provided superspeciality services in various disciplines. The subjects included 201 patients with Eisenmenger syndrome--diagnosed by a combination of echocardiography and a peripheral arterial oxygen saturation study and/or cardiac catheterisation with or without angiocardiography--worked up and followed up for variable duration over a period of 16 years from 1976 to 1992. One hundred nine patients were females and 92 were males--age of presentation varied from 3 months to 62 years (mean +/- standard deviation 19.23 +/- 12.62 years). A total of 12 different anatomic lesions were seen--the most common three being ventricular septal defect (33.33%), aterial septal defect (29.85%), and patent ductus arteriosus (14.23%). History, physical examination, chest skiagram and electrocardiogram established only the presence of pulmonary arterial hypertension except where differential cyanosis indicating ductus was discernible or the degree of splitting of second heart sound provided some clue to the level of shunt. Contrast echocardiography, completed in 25.4% established the level of shunt in all patients. In others the diagnosis was confirmed by cardiac catheterisation. Twenty patients died during a mean follow-up period of 54.6 +/- 54.47 months. Sudden cardiac deaths (30%), congestive heart failure (25%) and haemoptysis (15%) were the most predominant causes of death. Only one patient died during puerperium.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon pulmonary valvoplasty: factors determining short- and long-term results.

Balloon pulmonary valvoplasty was performed in 139 patients (age 2-44 years) with pulmonary valve stenosis. The right ventricular peak systolic pressure decreased from 137.1 +/- 46.8 mmHg to 76 +/- 51.3 mmHg (P < 0.001) and the right ventricle to pulmonary artery peak systolic gradient decreased from 116.3 +/- 49 mmHg to 54.4 +/- 51.9 mmHg (p < 0.001). There was no significant change in systemic artery systolic pressure. The right ventricular peak systolic pressure to systemic artery systolic pressure ratio decreased from 1.13 +/- 0.41 to 0.63 +/- 0.42 (P < 0.001). Patients with incomplete immediate relief of obstruction (right ventricle to pulmonary artery peak systolic gradient > 35 mmHg) had higher pre-dilatation right ventricular peak systolic pressure (161.1 +/- 45.3 mmHg vs. 93.9 +/- 38.8 mmHg, P < 0.001) and higher right ventricular peak systolic pressure to systemic artery systolic pressure ratio (1.31 +/- 0.42 vs 0.98 +/- 0.33, P < 0.001) pre-dilatation and were older (17.2 +/- 8.6 years vs. 12.8 +/- 9.7 years, P < 0.01). The residual right ventricle to pulmonary artery peak systolic gradients in the majority of patients were infundibular, which regressed at follow up even in patients who did not receive long-term oral beta blockers. Follow up catheterisation in 79 patients after 13 +/- 8.7 months showed a further fall in right ventricular peak systolic-pressure (P < 0.001) and right ventricle-to-pulmonary artery peak systolic gradient (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon angioplasty for native coarctation of the aorta in children and adults: factors determining the outcome.

Balloon angioplasty was performed in 46 patients (age 2-40 yr) with discrete native coarctation of aorta. Patients with associated patent ductus arteriosus, aberrant subclavian artery and aneurysms were excluded. The peak systolic gradient across the coarcted segment decreased from 52.1 +/- 18.5 mmHg to 18.6 +/- 14.8 mmHg (p less than 0.001), and the diameter of the coarcted segment increased from 3.6 +/- 1.7 mm/m2 to 9.1 +/- 3.2 mm/m2 (p less than 0.001). Follow-up haemodynamic and angiographic studies performed in 21 patients at 13.1 +/- 6.9 months after angioplasty, showed good results in 15 patients. Four patients undergoing haemodynamic study and 4 other patients undergoing noninvasive evaluation were graded as having bad results at follow-up. In 5 of these patients the poor results were due to primary failure of angioplasty in relieving the gradient, and three developed re-coarctation after initial fall in the trans-coarctation gradient. Four risk factors were identified on univariate analysis, which were associated with significantly larger residual gradients at follow-up: (1) size of isthmus/size of coarcted segment ratio less than 3.0; (2) size of post-coarctation descending aorta/size of isthmus ratio greater than 1.75; (3) size of coarcted segment after angioplasty/size of coarcted segment before angioplasty ratio less than 2.0; and (4) size of balloon/size of coarcted segment ratio less than 3.0. The presence of one or more risk factors was associated with bad late results. On multivariate analysis the ratio of balloon size/coarcted segment size was found to be the sole independent predictor of the late outcome (p less than 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

Right aortic arch with isolation of the left subclavian artery and bilateral patent ductus arterioses. A case report.

The surgical management of a case of right aortic arch with isolation of the left subclavian artery and bilateral patent ductus arterioses is described. A review of the 39 cases of isolation of the left subclavian artery reported in the literature revealed only one single case which on autopsy was found to have associated bilateral ductus arterioses. The case we report is the first in which the diagnosis was made during life and successful surgical correction was carried out.

Two dimensional and Doppler echocardiographic evaluation of double chambered right ventricle.

Two-dimensional and Doppler echocardiographic findings in 20 patients with double chambered right ventricle are described. All patients had the diagnosis established by cardiac catheterisation and confirmed at operation. Echocardiographic evaluation was done prior to surgical correction. Anomalous muscle bands in right ventricular cavity were detected in 16 patients. Doppler flow velocities in the right ventricular cavity suggested infundibular obstruction to blood flow at a low level in all 17 patients studied by Doppler echocardiography. Ventricular septal defects (11 patients), pulmonary stenosis (3 patients), and aortic regurgitation (3 patients) were detected accurately before operation by echocardiographic examination. Failure to detect the anomalous muscles in right ventricular cavity may occur in adult patients with poor anterior resolution and in those with severe right ventricular outflow obstruction and myocardial hypertrophy. Two dimensional echocardiography with Doppler flow analysis is useful in the evaluation and differential diagnosis of right ventricular outflow obstructions prior to invasive studies and surgical intervention.

Accuracy of continuous wave Doppler predicted gradient in patients undergoing balloon pulmonary valvoplasty.

14 patients who underwent balloon valvoplasty had trans-pulmonic gradient evaluated by continuous wave Doppler echocardiography. Maximum systolic gradients measured from Doppler flow velocities were correlated with peak to peak gradient obtained at cardiac catheterisation. Prior to valvoplasty, there was good correlation between the Doppler maximum gradient (92.85 +/- 34.7mm Hg) and the peak to peak catheter gradient (105.57 +/- 56.60 mm Hg), (r = 0.91, p = less than 0.001). Immediately after balloon valvoplasty, the maximum Doppler gradient did not correlate with the peak to peak catheter gradient (r = 0.33, p = NS). Exclusion of patients with infundibular gradients improved the correlation coefficient between the Doppler maximum and peak to peak catheter gradient to 0.69. At late restudy following valvoplasty, when regression of infundibular stenosis was noted in 6 out of 8 patients, the Doppler maximum and catheter peak to peak gradient had excellent correlation (r = 0.97, p = less than 0.001). In patients with lone valvular gradient immediately following valvoplasty and at late restudy, maximum Doppler gradients correlated well with catheter gradients in 14 estimations (r = 0.66, p = less than 0.01). This study shows that the non-invasive quantification of pulmonary valve stenosis can be reliably undertaken, using continuous wave Doppler echocardiography before balloon valvoplasty and during follow-up, after the procedure when the infundibular stenosis has regressed. The presence of an infundibular gradient immediately after balloon dilatation makes the Doppler prediction less reliable.

Two-dimensional and Doppler echocardiographic study of coronary arteriovenous fistulas.

Sixteen patients with coronary arteriovenous fistula (CAVF) were studied by two-dimensional echocardiography (2DE). Of these 12 had Doppler studies. In all, the diagnosis of CAVF was confirmed by aortic root or selective coronary angiography. In 8 patients, the 2DE findings suggested CAVF. Five patients had dilated main coronary arteries and in 4 patients abnormal fistulous channels were identified. Two had diastolic flutter of the tricuspid valve and one had spontaneous contrast in the right atrium. In 11 out of 12 patients, Doppler analysis picked up a continuous flow (to the right atrium in 5, right ventricle in 4 and to both ventricles in 2 patients) prior to the cardiac catheterisation. We conclude that noninvasive diagnosis of CAVF can be made with the combined use of two-dimensional and Doppler echocardiography in most of the patients.

Assessment of severity of aortic stenosis by continuous wave Doppler echocardiography.

Fifty consecutive patients with aortic stenosis were evaluated by continuous wave Doppler echocardiography for assessment of the transaortic gradient. The Doppler derived gradients were compared with the gradients measured at cardiac catheterisation. Excellent correlation was found between the Doppler and catheterisation findings for the maximum instantaneous gradient (r = 0.92) and the mean systolic gradient (r = 0.84). The maximum, midsystolic and late systolic Doppler gradients also showed a good correlation with the peak to peak catheter gradient. The maximum Doppler velocity however, showed overestimation of the peak to peak gradient in the presence of mild aortic stenosis (predictive accuracy 86%). The midsystolic Doppler velocity showed the highest predictive accuracy (94%) for the detection of severe aortic stenosis. No case of severe aortic stenosis was missed by Doppler using either the maximum or midsystolic Doppler velocity. These findings indicate that continuous wave Doppler ultrasound provides a reliable estimate of the gradient in patients with aortic stenosis.

Blade and balloon atrial septostomy for creation of an adequate interatrial communication.

During a 7 year period from 1984 to 1991, 100 infants underwent either balloon atrial septostomy (BAS) (n = 92) or blade septostomy (BLS) (n = 8). The indication was complete transposition of the great arteries in all the patients. The mean age in the BAS group was 1.8 +/- 1.5 months while that in the infants requiring BLS due to a thick septum was 3.03 +/- 2.29 months. The degree of improvement in arterial oxygen saturation in both groups was satisfactory -27.16 +/- 14.06% in the BAS group vs 23.5 +/- 12.18% in the BLS group. There were no procedure related deaths in the BLS group, through only monoplane fluoroscopy was used in 6/8 patients. Three patients however died following balloon septostomy. We conclude that BLS is a safe and effective alternative to surgical septostomy when performed with due care. Two dimensional echocardiography during BAS enhances the speed and safety of the procedure and helps to identify patients who may require BLS due to a thick interatrial septum.

Primary pulmonary hypertension: natural history and prognostic factors.

A retrospective cohort study was carried out in 61 patients (30 males, 31 females, age 24.6 +/- 11.8 years) with primary pulmonary hypertension diagnosed by strict clinical and hemodynamic criteria, to obtain an understanding of the natural history and prognostic markers. While 15 patients were alive, 46 patients (76%) had expired during the follow up period. Two, five and ten years survivals were 48%, 32% and 12% respectively. Median survival duration from time of diagnosis was 22 months. The survivors had significantly higher age of onset, cardiac index and significantly lower right atrial mean pressure, right ventricular end diastolic pressure, cardiothoracic ratio from chest rontgenogram and calculated pulmonary vascular resistance as compared to non survivors. While pulmonary artery systolic pressure was not significantly different, pulmonary artery diastolic and pulmonary artery mean pressures were significantly lower in survivors than in non-survivors. Lower New York Heart Association class, right atrial mean pressure < or = 7 mm Hg, right ventricular end diastolic pressure < or = 10 mmHg, cardiac index > 2.5 L/min/m2, pulmonary arterial oxygen saturation > 60%, were associated with significantly longer survival. The degree of pulmonary arterial hypertension had an indirect prognostic effect through the above parameters. Vasodilator therapy did not significantly alter the outcome of patients with primary pulmonary hypertension.

Thrombolytic therapy for prosthetic cardiac valve thrombosis.

During 1992-93 12 patients (5 males, 7 females) with thrombosed prosthetic cardiac valves were treated with streptokinase on 13 occasions (one patient with prosthetic tricuspid valve had two thrombotic episodes). Their age ranged from 14 to 52 years (median 39). Two valves were in aortic position, six in mitral and four in tricuspid position. Eight were Bjork-Shiley prosthesis, three were Medtronic Hall valves and one was a St. Jude Valve. Timing of prosthetic valve thrombosis ranged from 3 months to 12 years after valve replacement surgery. Duration of symptoms due to valve thrombosis ranged from 1-4 months with tricuspid valve thrombosis and 1-14 days with left sided valve thrombosis. Five were in functional class II and four each were in functional class III and class IV. All patients were evaluated by echo Doppler and cine fluoroscopy. Loading dose of streptokinase was 2.5 lakh units in 4 patients and 1 lakh units in 9 patients. Maintenance infusion was at 1000 units/Kg/hour in 11 patients and 1 lakh units/hour in 2 patients. Duration of streptokinase infusion ranged from 3 hr to 38 hr. Thrombolytic therapy was successful (clinical, echo Doppler and fluoroscopy) in 12 out of 13 cases (92%). It was unsuccessful in a patient with valve at tricuspid position in whom infusion had to be stopped after 24 hour due to bleeding gums. One patient developed intracerebral bleed and expired. In conclusion streptokinase therapy is useful for prosthetic cardiac valve thrombosis.