Wide complex rhythm in a well neonate: Where are the P waves?

A neonate was seen for an evolving broad QRS complex rhythm initially captured at birth as intermittent escape beats on electrocardiogram. Continuous monitoring recorded features mimicking pre-excitation, but closer analysis revealed a regular broad QRS complex rhythm with isorhythmic atrioventricular dissociation, favouring a ventricular source. Treatment with flecainide and propranolol achieved successful control of the incessant arrhythmia with improvement in cardiac function on echocardiogram.

Management of rheumatic aortic valve disease using the Ozaki procedure with autologous pericardium: a case report.

BACKGROUND: Rheumatic valve disease (RVD) is the most common cause of cardiovascular death in low-middle income nations. Surgical aortic valve (AV) interventions for RVD, especially in children, have proven problematic with graft failure, relapse, and poor compliance with anticoagulation. A novel technique involving neocuspidization of the aortic annulus using autologous pericardium to construct new AV leaflets (the Ozaki procedure) has shown promising outcomes in children with congenital AV disease; however, there are no previous recorded cases using this technique in children with RVD. CASE SUMMARY: We present the case of a 15-year-old male presenting with exertional angina and dyspnoea with a background of previous rheumatic fever. Echocardiography had shown a regurgitant tricuspid AV, left ventricular dilatation with mitral valve leaflet tethering. The patient underwent the Ozaki procedure for his AV regurgitation and was discharged following an uneventful post-operative recovery. The patient had full resolution of symptoms following the procedure and remains well 3 years following his operation. DISCUSSION: This case highlights that good outcomes with the Ozaki procedure in RVD are possible 3-years post-operatively and should prompt future studies to evaluate the procedure as a surgical option for paediatric patients in this clinical context. Additionally, the Ozaki procedure may also provide a cost-effective surgical technique requiring minimal additional operative resources and reduced follow-up demand, which would be critical in low-resource clinical settings where RVD is prevalent.

Sildenafil in the management of the failing Fontan circulation.

BACKGROUND: Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn. Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically. AIM: Present our experience with sildenafil treatment in patients with a failing Fontan circulation. PATIENTS AND METHODS: Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008. RESULTS: Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1-2 milligrams per kilogram 3-4 times per day. Protein-losing enteropathy and alpha-1-antitrypsin levels improved in all three patients on sildenafil treatment. One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration. There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years. CONCLUSIONS: Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.

Fate of pulmonary arteries following Norwood Procedure.

OBJECTIVE: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. METHODS: Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock-Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). RESULTS: Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60+/-3%, 52+/-4% and 50+/-4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR 8.3, 5.3-13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98+/-1%, 72+/-4% and 58+/-6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6-9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. CONCLUSIONS: CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.

Severe left main coronary artery stenosis with abnormal branching pattern in a patient with mild supravalvar aortic stenosis and Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenoses of medium- and large-sized arteries including the coronary arteries. Cardiac manifestations are often the initial reason for referral and careful cardiovascular assessment is important as coronary artery involvement confers a significant anesthetic risk and may be associated with ischemia and resultant ventricular dysfunction. Here we review the literature and describe a 2-year-old boy with evolving clinical features of WBS. He presented to our pediatric cardiology department for a routine assessment of peripheral pulmonary branch stenosis. A 12-lead electrocardiogram showed changes consistent with left ventricular ischemia and a two-dimensional echocardiogram showed reduced left ventricular function and mild supravalvar aortic stenosis. Subsequent cardiac catheterization diagnosed severe left main coronary artery stenosis. Deteriorating ventricular function secondary to acute ischemia postcatheterization required intensive care treatment from which the patient did not recover. This case report highlights the necessity of careful cardiology assessment without delay in patients with a suspicion of WBS. Isolated coronary stenosis though rare in WBS should be considered in the presence of ischemia or reduced ventricular function. Larger case series are needed to further characterize the correlation between WBS and acute coronary events.