Isolated cleft mitral valve with severe regurgitation resulting in a left inferior pulmonary vein varix diagnosed from cardiovascular magnetic resonance imaging.

Isolated cleft mitral valve (ICMV) is a rare entity not known to be related to pulmonary atresia with ventricular septal defect (PA-VSD). This report describes the use of cardiovascular magnetic resonance (CMR) imaging to diagnose ICMV in a patient with repaired PA-VSD who presented with incidental severe mitral regurgitation (MR) on follow-up echocardiography. An associated pulmonary varix secondary to the severe MR also was shown by CMR.

Influence of surgery on aortic valve prolapse and aortic regurgitation in doubly committed subarterial ventricular septal defect.

Doubly committed subarterial ventricular septal defects should be closed surgically once aortic valve deformity is present before the onset of aortic regurgitation.

Outcome of surgical closure of doubly committed subarterial ventricular septal defect.

BACKGROUND: From 1986 to March 1997, 128 patients diagnosed to have doubly committed subarterial ventricular septal defects (VSD) were reviewed. Patients with aortic regurgitation (AR), and aortic valve (AV) deformity or a large left-to-right shunt across the VSD were offered operation. Forty-five patients (27 men, 18 women) agreed to surgical closure of their VSDs. METHODS: Thirty-eight patients had VSD closure alone, and 7 had an additional AV repair. Other associated defects corrected at operation were closure of atrial septal defects, closure of other ventricular septal defects, ligation of patent ductus arteriosus, and repair of ruptured sinus Valsalva aneurysm. RESULTS: There was no mortality nor major morbidity associated with operation. In the 26 patients with AR and AV deformity preoperatively, valve repair was performed in 6 patients. The condition of AR improved in 4, and remained unchanged in 22 patients. In the 10 patients with a deformity of the AV and no AR preoperatively, the condition remained unchanged in 5 patients, from whom 1 had valve operation, but progressed in 5 patients postoperatively at a mean follow-up of 6.4 years. In 9 patients with no deformity of the AV and no AR preoperatively, there was no postoperative AR and no progress of valve deformity. CONCLUSIONS: Excellent results were obtained with VSD closure and AV repair. Surgical closure of VSD, if performed before the onset of AV deformity, may prevent progressive AR. If AV repair is performed after the onset of AV deformity, progressive AR may not always be prevented.

Abnormal electrocardiographic patterns in renal failure. Ventricular tachycardia.

A 9-year-old girl was diagnosed to have renal failure. However, her family has not been compliant with medical treatment and opted for traditional therapy instead. She was admitted in an ill state with fluid overload. What is the ECG diagnosis?

Cardiac dysrrhthmia in systemic disease.

This is an ECG of a 6-year-old girl. She presented to us for management of epilepsy. Magnetic resonance imaging (MRI) of the brain showed multiple lesions consistent with cortical and subcortical tubers. There were also achromic spots on her skin and echocardiography demonstrated a rhabdomyoma near the right ventricular outflow tract. A diagnosis of tuberous sclerosis, an autosomal-dominantly inherited condition, was made. What is abnormal about the ECG?

Paediatric pacemaker implant using the transvenous endocardial approach.

The advent of the pacemaker has opened a whole new dimension to management in cardiology. Although its use has been well described in adults, its role in paediatric cardiology has proven to be equally exciting and challenging. We describe a case of a child with complex cyanotic heart disease who had an insertion of a cardiac pacemaker via the transvenous route, one of the youngest to be performed locally. This article also highlights the pertinent features of paediatric pacemaker therapy, including its indications and implantation technique.

A case report on the perinatal management of a 30-week preterm baby with congenital complete heart block.

INTRODUCTION: Congenital complete heart block is an uncommon condition in the newborn, but is known to occur with maternal systemic lupus erythematosus. CLINICAL PICTURE: This paper presents one such baby with complete heart block who was born premature (after a gestation of 30 weeks) and weighing 759 g. TREATMENT: Continuous isoprnaline infusion was initially used to support the baby while her other neonatal problems were treated. A Medtronics VV1 pacemaker was subsequently inserted to maintain a heart rate that would be more physiologically acceptable for the patient. OUTCOME: This baby is currently thriving well, having been followed up for one year. CONCLUSIONS: The management issues, encompassing maternal and neonatal problems, and a review of current literature on this condition are discussed.

Liver transplantation in a child with severe hypercholesterolaemia in Alagille syndrome.

INTRODUCTION: Liver transplantation is a curative treatment modality in children with end stage liver disease in Alagille syndrome. CLINICAL PICTURE: We report a 3-year-old child with this condition who had severe hypercholesterolaemia, pruritus and extensive xanthomatosis. TREATMENT: Liver transplantation was performed in this patient. OUTCOME: He recovered well with normalisation of his lipid profile. This procedure also resulted in resolution of the disfiguring xanthomatosis.

A case series of six children with primary pulmonary hypertension.

INTRODUCTION: Primary pulmonary hypertension is an uncommon but serious disease in children. Management is difficult despite recent advances in pharmacotherapy. CLINICAL PICTURE: We reviewed patients with this condition with respect to their presenting symptoms, investigations, treatment and outcome. TREATMENT: These children were treated with individualised combinations of oxygen, diuretics and calcium antagonists. OUTCOME: In our follow-up of 5 children, 4 had died. CONCLUSION: This condition is irreversible and progressive with a high mortality rate. A better understanding of, and research into, the pathogenesis would hopefully lead to the formulation of improved therapeutic strategies for this condition.

Initial experience of radiofrequency catheter ablation of supraventricular tachycardia in paediatric patients.

INTRODUCTION: The management of supraventricular tachycardia (SVT) in paediatric patients until recently has frequently been pharmacologic therapy, but this approach suffers from the drawbacks of treatment failure, development of drug intolerance and/or side-effects. AIM: In keeping with recent advances in paediatric cardiology, we share our experience with radiofrequency catheter ablation as an alternative and definitive modality of therapy. MATERIALS AND METHOD: 4 young patients with recurrent SVT underwent electrophysiologic study followed by radiofrequency ablation of the accessory pathways. RESULTS: Resolution of symptoms was achieved in all patients and no major complication was encountered. CONCLUSIONS: The ability to ablate permanently the reentrant circuit responsible for SVT has now permitted cure by non-surgical means, and is an important alternative to drug therapy in the management of SVT in children.

Hepatopulmonary syndrome: a rare complication of chronic liver disease in children.

An 11-year-old boy with congenital hepatic fibrosis presented with cyanosis at the National University Hospital. Echocardiogram revealed a structurally normal heart with good ventricular function. A pulmonary cause of his cyanosis was suggested on macroaggregated albumin scan and selective pulmonary artery angiogram. Arterial hypoxaemia secondary to intrapulmonary arteriovenous shunting in chronic liver cirrhosis can lead to permanent cyanosis. The potential for a complete reversal of this condition after liver transplantation indicates that arterial hypoxaemia, rather than being a contraindication, should be a reason for early liver transplantation.

The Polarprobe--emerging technology for cervical cancer screening.

The Polarprobe is a portable non invasive electronic device designed for the detection of cervical precancer and cancer. It measures both electrical and optical properties of cervical tissue to allow a real time comparison with a databank of previously determined cervical tissue types. The need for additional tests to augment or even replace the Papanicolaou smear has partly prompted its development. Indeed it has been shown to be associated with less pain and anxiety than the smear and has the capability of encouraging women to attend for screening. Some of the preliminary clinical trials on the Polarprobe are reported as well as the ongoing developments and modifications to the device.

Bidirectional cavopulmonary anastomosis.

The Fontan operation has been used to palliate patients with a functional single ventricle. In many such patients the operative risk for a Fontan procedure is high. The bidirectional cavopulmonary anastomosis (BCPA) is a useful intermediate palliative procedure before Fontan correction. It may reduce the deleterious sequelae of chronic hypoxaemia and long-term ventricular overload, thus yielding a more suitable Fontan candidate. We reviewed our experience of 9 patients with univentricular hearts who were at the time of the BCPA not considered suitable candidates for conventional Fontan operation. Two patients had double inlet right ventricle (DIRV) with pulmonary stenosis (PS) and ventricular septal defect (VSD), 2 had univentricular heart, 4 had tricuspid atresia (TA) and hypoplastic right ventricle (RV), 1 patient had atrioventricular septal defect (AVSD) with double outlet right ventricle (DORV) with hypoplastic RV. Two of the 9 patients had bilateral superior vena cavae. All except one had undergone prior palliative operations. The mean age at BCPA was 4.7 years (range 1.5 years to 6 years) and the mean weight at surgery was 15 kg (range 7 to 22 kg). There were no operative deaths. The mean hospitalization stay was 8.5 days (range 5 to 13 days) with a mean follow up of 14 months. Median oxygen saturation improved from 78.7 +/- 6.3% to 85.9 +/- 3.9%. The BCPA is a useful palliative procedure to stage Fontan type reconstruction in selected patients with univentricular hearts.

Coarctation of the aorta: nonsurgical treatment using stent implantation.

INTRODUCTION: Coarctation of the aorta (CoA) accounts for 5%-8% of all congenital heart defects. If left untreated, most patients with significant CoA will have varying degrees of morbidity (e.g. hypertension, stroke, collateral formation and ventricular hypertrophy), possibly even mortality. Traditionally, treatment for this condition is surgical. Herein, we report stenting during catheterisation as an alternative nonsurgical treatment option for patients with CoA, and present the treatment outcomes of patients who underwent this treatment option. METHODS: We retrospectively reviewed four patients (2 men and 2 women; age range 20-41 years) who underwent CoA stenting under general anaesthesia for the treatment of native CoA or restenosis of CoA at our institution. Three patients had a 40-mm Palmaz stent inserted, while one had a 39-mm Cheatham-Platinum covered stent inserted. Angiography and measurement of pressure gradients were performed before and after stent implantation to ensure good treatment outcomes. RESULTS: The patients' treatment outcomes were good, with a significant reduction in pressure gradients across the narrowed segments. Angiography showed relief of CoA. The patients were followed up for 1-3 years, during which no complications were noted. CONCLUSION: This is the first reported series in Singapore on the nonsurgical treatment of CoAs in adult patients using stents during interventional cardiac catheterisation. This less invasive procedure may lead to a new paradigm shift with regard to the treatment of CoA.