Effect of anti-heart failure therapy on diastolic function in children with single-ventricle circulations.

BACKGROUND: Children with functionally univentricular circulations have chronic volume loading of the systemic ventricle, potentially affecting ventricular function. Medications including angiotensin-converting enzyme inhibitors and ß-blockers are used to treat ventricular dysfunction, despite limited evidence of their efficacy in this population. OBJECTIVE: To determine the effects of angiotensin-converting enzyme inhibitors on elevated filling pressures in children with single ventricle physiology. METHODS: We performed a single-centre, retrospective review of patients with single ventricle physiology who underwent multiple cardiac catheterisations between 1991 and 2013. Study population comprised of patients who commenced or had optimised dosing of angiotensin-converting enzyme inhibitors between assessments in response to high ventricular filling pressures. Patients undergoing interventions influencing loading conditions between assessments were excluded. RESULTS: A total of 17 patients were identified, with dominant morphologic right ventricle in eight patients (47.1%). Among them, 11 (64.7%) were pre-Fontan and six (35.3%) were post-Fontan completion. Median inter-assessment interval was 9.4 months (range 7.3-19.1). There was a reduction in end-diastolic pressure from 13 to 10 mmHg (p=0.002), mean pulmonary artery pressure from 16 to 13 mmHg (p=0.049), and mean atrial pressure from 12 to 9 mmHg (p=0.001). There was one cardiac transplant, and there were no patient deaths at median follow-up after 31 months. CONCLUSIONS: We observed a reduction in ventricular end-diastolic pressure, pulmonary artery pressure, and mean atrial pressure following treatment with angiotensin-converting enzyme inhibitors in patients with single ventricle physiology. Our study provides insights into the potential impact of anti-heart failure therapy in single ventricle circulations and calls for larger, controlled studies to assess for a therapeutic response.

Imaging and percutaneous occlusion of a large aneurysm of the ductus arteriosus in an infant with Loeys-Dietz syndrome.

Loeys-Dietz is a multisystem congenital syndrome that comprises craniofacial and cutaneous abnormalities as well as structural cardiac defects. One of its key pathological features is an aggressive widespread vasculopathy that can manifest as aortic or cerebral aneurysms, which is prone to dissection and rupture. We report a case of a large aneurysm of the ductus arteriosus in a patient with Loeys-Dietz syndrome, successfully occluded by interventional catheterization.

The sinister course of an intramural right coronary artery.

We report the case of an adolescent who was presented with long-standing exertional symptoms, and was diagnosed with an anomalous right coronary arterial origin arising above the commissural junction between the left and right aortic sinus, with inter-arterial and intramural compression. The precise origin of this lesion outside the aortic sinuses is unusual, and multi-detector computed tomography gave excellent definition and spatial resolution of the anomalous origin and course. It is crucial to have a high index of suspicion of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery.