Usefulness of Doppler derived end diastolic flow gradient across the patent ductus arteriosus in selecting coils for ductal occlusion.

Transcatheter closure of patent ductus arteriosus (PDA) with coils is accepted as an alternative to surgical ligation. We evaluated whether flow gradient across PDA, obtained by Doppler echocardiography, can aid in selecting coils for percutaneous ductal occlusion. 79 consecutive patients with PDA, who underwent successful percutaneous coil occlusion were retrospectively reviewed. Patients with other structural heart disease and pulmonary hypertension with right-to-left shunt were excluded. Echocardiogram and cardiac catheterization were done in all patients. Gianturco (Occluding Spring Emboli; Cook, Bloomington, IN) non-detachable coils of 0.038 and 0.052-inch core sizes were used for ductal occlusion. Trough diastolic gradient was correlated with the size and the number of coils used. Mean age was 8.6 years (range 1.3 to 27 years); 24 males and 55 females; PDA diameter ranged from 1.3 to 4.5 mm. Number of coils used varied from 1 to 4. Echocardiography measured PDA size was 2.5 ± 0.6 mm and significantly differed from angiographically measured size 2.9 ± 0.6 mm (P = 0.05). End diastolic gradient below 38 mmHg predicted use of multiple coils or coils with larger surface area. End diastolic gradient correlated inversely with total surface area of the coils, which indirectly predicted size and number of coils. Thus, the prediction of the size and the number of coils for PDA occlusion can be assisted by the trough diastolic gradients of PDA.

Electrocardiographical case. J wave and presyncope in a middle-aged woman.

A 46-year-old woman presented with three episodes of presyncope in the previous two days. Electrocardiogram (ECG) showed sinus rhythm, relatively short QT interval (QTc of 340 msec) and prominent J-waves in the inferior leads. Biochemical evaluation revealed serum calcium of 17.4 mg/dL, phosphorus of 2.3 mg/dL and alkaline phosphatase of 533 IU/ml. She was managed with saline diuresis followed by elective parathyroidectomy. ECG became normal with the disappearance of J-waves after correction of hypercalcaemia. She was asymptomatic at six months of follow-up. The presence of J-waves (Osborne waves) in hypercalcaemia has rarely been reported.

Cardiac malformations are increased in infants of mothers with epilepsy.

We aimed to ascertain the prevalence of cardiac malformation (CM) and its association with antenatal exposure to an antiepileptic drug (AED) in infants of mothers with epilepsy (IMEs). Women with epilepsy (WWE) are enrolled in Kerala Registry of Epilepsy and Pregnancy (KREP) in the prepregnancy or early pregnancy period and are followed up with a standard protocol until the IMEs are 6 years old. At 3 months postpartum, a cardiologist, blinded to the AED exposure, carried out a clinical examination and echocardiography on all live-born babies. Patent foramen ovale (PFO) and interatrial septal defects of < 5 mm in size were excluded from CM. Details of maternal epilepsy, folate usage, AED exposure in the first trimester, and newborn characteristics were abstracted from the records of the KREP. We examined 462 babies. Maternal epilepsy was generalized in 201 (43.50%) or localization related in 241 (52.2%). The AED exposure was monotherapy in 262 (56.7%)--carbamazepine (112), valproate (71), phenobarbitone (43), phenytoin (31), and clonazepam (2)--and polytherapy in 126 (27.3%). Seventy-four infants (16.01%) had no AED exposure. There were 36 infants with CM (7.8%; 95% confidence interval: 5.5-10.6). CMs included atrial septal defect (26; 72.2%), tetrology of Fallot (3; 8.3%), patent ductus arteriosus and pulmonic stenosis (2 each; 5.6%), and ventricular septal defect, tricuspid regurgitation, transposition of great arteries (1 each; 2.8%). CMs were significantly more for IMEs with premature birth (p < .003). There was no association between CM and maternal age, epilepsy syndrome, seizure frequency during pregnancy, and folate use. CMs were more frequent with polytherapy (13; 10.3%) compared to monotherapy (17; 6.5%). Those with valproate exposure had a trend (not statistically significant) toward higher frequency of CM compared to IMEs on other AEDs as monotherapy.

Intra-atrial extension of Wilms' tumor.

Echocardiography in a 4-year-old boy, with nephroblastoma of the left kidney, revealed a large homogenous mass in right atrium extending from inferior vena cava, and protruding through tricuspid valve into right ventricle during diastole. Ultrasonography revealed the contiguous spread of the tumor through renal vein with near total caval occlusion. The patient had a fatal outcome before definite treatment could be started. Intracardiac extension of infradiaphragmatic tumors through caval route, although infrequent, can be seen with renal cell carcinoma, Wilms' tumor, hepatoma, lymphoma, and uterine and adrenal tumors. Detection of a mass in right atrium in a child should alert the echocardiographer about the possibility of caval spread from a renal neoplasm.

Right pulmonary artery-to-left atrial fistula: a differential diagnosis of cyanosis without structural heart disease.

We present the case of a 19-year-old male who presented with late-onset cyanosis and a normal cardiovascular system examination. Contrast echocardiography revealed a right-to-left shunt. A right pulmonary artery-to-left atrial fistula was seen on the angiogram, which was surgically corrected. Early intervention is indicated to prevent complications in patients with this condition.

Single coronary artery from right aortic sinus with septal course of left anterior descending artery and left circumflex artery as continuation of right coronary artery: a hitherto unreported coronary anomaly.

A single coronary artery (SCA) in the absence of structural heart disease is a rare coronary anomaly and is often detected incidentally during coronary angiography. We report a hitherto undescribed type of SCA originating from the right sinus of Valsalva, with the left anterior descending artery having a septal course and the right coronary artery continuing as the left circumflex artery, which was incidentally detected in a 73-year-old female.

Induction of atrioventricular nodal reentry tachycardia with intravenous adenosine.

Adenosine, used to terminate paroxysmal supraventricular tachycardia (SVT), is often useful in understanding the mechanism of tachycardia. This case report describes induction of SVT with adenosine in a 36-year-old man presenting with recurrent palpitations. After a short run of conduction via both slow and fast pathways, SVT was induced following a long PR interval. The long PR interval resulted by conduction via the slow pathway due to the preferential conduction block by adenosine over fast pathway. The notching at the terminal part of QRS during antegrade slow pathway conduction and during tachycardia indicated activation of the atrium via retrograde fast pathway. This electrocardiographical feature confirmed the mechanism of the tachycardia as atrioventricular nodal reentrant tachycardia.

Ebstein's anomaly, Wolff-Parkinson-White syndrome and rheumatic mitral stenosis: role for combined electrophysiological and surgical management.

The coexistence of rheumatic mitral stenosis, Ebstein's anomaly and Wolff-Parkinson-White syndrome is an uncommon entity. To our knowledge, the successful management of this combination of lesions has not been previously described. We report a 23-year-old woman with the combination of these abnormalities. She was managed with preoperative electrophysiological study, followed by mitral valve replacement and Danielson's repair of tricuspid valve. The management issues involved are discussed in detail.

Absent pulmonary valve and origin of left pulmonary artery from ascending aorta.

A rare combination of tetralogy of Fallot, absent pulmonary valve and absent left pulmonary artery is described. The clinical and angiographic profile and the impact on management are discussed. The embryology is outlined.

Star in the heart.

In a 50 year old woman, transthoracic echocardiography showed a left atrial mass. Transoesophageal echocardiography delineated its attachment. Additionally, cystic spaces were seen arranged concentrically in the shape of a star in the centre of the tumour. Surgical excision followed. Histopathological examination confirmed myxoma with areas of haemorrhage and necrosis. This case highlights the acoustic property of myxomas in a rare and beautiful manner and emphasises the superior transoesophageal imaging of myxomas.

Calcific stenotic jump graft.

A young lady with post-subclavian coarctation and cranial hypertension is discussed. She had a jump graft from left subclavian artery to descending aorta, and presented 18 years later with hypertension, calcification of the graft with a gradient of 40 mmHg across it. In the meantime, she also developed moderate aortic regurgitation on a bicuspid aortic valve. Management strategies including trans-catheter options are discussed. We present our reservations on trans-catheter options in an occluded dacron graft.

Coronary artery fistula in children and adults: a review of 25 cases with long-term observations.

We studied 25 patients with coronary artery fistula between 1976 and 1994. Age ranged from 1 to 58 years. Twelve patients were symptomatic; seven had dyspnoea, four had angina, one had palpitation and one had syncope. Coronary arteries in four. Coronary artery fistula drained into right ventricle in 11, right atrium in nine, pulmonary artery in four and left ventricle in two. The Qp/Qs ranged from 1.0 to 2.6 with a mean of 1.39 +/- 0.38. Five patients had associated cardiac anomalies. Two had atrial septal defects, one had patent ductus arteriosus, one had atresia of proximal right coronary artery and in one patient, the right coronary was arising from left coronary artery. Five patients underwent surgery without any operative mortality. Thirteen patients were followed-up medically for a mean period of 6.1 +/- 5.1 years. There were no complications related to coronary artery fistula during follow-up. In one patient coronary artery fistula closed spontaneously.

Bidirectional Glenn shunt: a step in the right direction.

Bidirectional Glenn (BDG) shunt was carried out in 14 patients (age range 1.5-22 years; mean 9.3 years) for a variety of congenital cyanotic heart diseases with decreased pulmonary blood flow. Postoperative cardiac catheterisation was carried out in 10 patients including successful balloon angioplasty of the shunt and LPA in one patient. There was a significant improvement in oxygen saturation and a drop in haematocrit level at follow-up. Doppler echocardiography studies in 13 patients revealed functioning shunts in all cases with low velocity continuous flow. Thus BDG is a useful palliative procedure and could be undertaken as the first stage of total cavopulmonary connection in high-risk Fontan groups where biventricular repair is not feasible.

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.