Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease.

BACKGROUND: Adults with pulmonary hypertension associated with congenital heart disease (PH-CHD) often have residual shunts. Invasive interventions aim to optimise pulmonary flow and prevent right ventricular failure. However, eligibility for procedures strongly depends on the adaptation potential of the pulmonary vasculature and right ventricle to resultant circulatory changes. Current guidelines are not sufficiently applicable to individual patients, who exhibit great diversity and complexity in cardiac anomalies. METHODS AND RESULTS: We present four complex adult PH-CHD patients with impaired pulmonary flow, including detailed graphics of the cardiopulmonary circulation. All these patients had an ambiguous indication for shunt intervention. Our local multidisciplinary Grown-Ups with Congenital Heart Disease team reached consensus regarding a patient-tailored invasive treatment strategy, adjacent to relevant guidelines. Interventions improved pulmonary haemodynamics and short-term clinical functioning in all cases. CONCLUSIONS: Individual evaluation of disease characteristics is mandatory for tailored interventional treatment in PH-CHD patients, adjacent to relevant guidelines. Both strict registration of cases and multidisciplinary and multicentre collaboration are essential in the quest for optimal therapy in this patient population.

Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient.

BACKGROUND: Pulmonary hypertension (PH) is a leading cause of death in systemic sclerosis (SSc) patients. The current study assessed the ability of the ECG-derived ventricular gradient (VG-RVPO) to detect PH and predict all-cause mortality in PH patients with subtypes of SSc differing in the extent of multi-organ involvement. METHODS: ECGs were obtained from 196 patients with limited and 77 patients with diffuse SSc included from our screening programme on cardiac complications. The association of the VG-RVPO with (1) the presence of PH, (2) conventional screening parameters and (3) survival in PH patients was assessed. RESULTS: In limited SSc patients an elevated VG-RVPO corresponded with the presence of PH (-5±12 mV.ms vs -22±16 mV.ms, P<0.01), correlated significantly with conventional screening parameters and had a better diagnostic performance than the presence of a right heart axis (AUC 0.81 vs 0.60; P=0.04). These differences were not observed in patients with diffuse SSc. An elevated VG-RVPO was associated with decreased survival in all SSc patients with PH (3 year survival 30% vs 64%, P=0.02). CONCLUSION: An elevated VG-RVPO is associated with PH in limited SSc patients and with decreased survival in all SSc patients with PH.

Pulmonary hypertension: the role of the electrocardiogram.

A 54-year-old female was referred to our centre for further evaluation of recently established severe pulmonary hypertension. Six months prior to presentation to the cardiologist of the referring centre, the patient had first experienced exertional dyspnoea. At the time of presentation to the referring cardiologist, the patient's ECG showed signs of an increased right heart load. Interestingly, this patient had undergone a thorough cardiac evaluation in the referring centre seven years before when she presented with severe hyperthyroidism. At that time there were no symptoms or signs of pulmonary hypertension on ECG, echocardiography, or at heart catheterisation. Thorough evaluation in cooperation with the referring centre demonstrated that this patient was suffering from idiopathic pulmonary arterial hypertension, a rare form of pulmonary hypertension. We conclude this report with a discussion on the potential use of the ECG for the diagnosis of increased right heart load. (Neth Heart J 2008;16:250-4.).