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Background: Pulmonary arterial hypertension (PAH) is characterized by a pulmonary capillary wedge pressure (PCWP) of ≤15 mm Hg, given a normal left ventricular filling pressure (LVFP). However, recent studies have shown that, in PAH patients, diagnosis based on PCWP can erroneously classify a significant number of patients compared with diagnosis based on left ventricular end-diastolic pressure (LVEDP). Therefore, we sought to compare the diagnostic accuracy of end-expiratory PCWP and LVEDP measurements in patients suspected of having pulmonary hypertension (PH)., Methods: We reviewed the hemodynamic data from 122 patients suspected of having PH who underwent simultaneous right- and left-side heart catheterizations at a PH referral center from 2006 to 2011., Results: PH was diagnosed in 105 patients, 79% of whom (n = 83) showed a pre-capillary pattern according to the LVEDP measurement. Ninety percent of patients with PCWP ≤15 mm Hg were correctly classified as having pre-capillary PH. However, 39% of patients with a PCWP >15 mm Hg had LVEDP ≤15 mm Hg and would have been erroneously diagnosed with pulmonary venous hypertension based on their PCWP measurements alone. The sensitivity and specificity was 0.89 and 0.64, respectively. A Bland-Altman analysis of the PCWP and LVEDP measurements revealed a mean bias of 0.3 mm Hg with 95% limits of agreement of -7.2 to 7.8 mm Hg., Conclusions: A PCWP ≤15 mm Hg was found to be a reliable indicator of normal LVFP in pre-capillary PH patients. When measured properly and analyzed in the clinical context, PCWP is a valuable tool for accurate diagnosis of PAH., (© 2014 International Society for Heart and Lung Transplantation Published by International Society for the Heart and Lung Transplantation All rights reserved.)

Background: Haemodynamic responses to exercise are related to physical impairment and worse prognosis in patients with pulmonary arterial hypertension (PAH). It is clinically relevant, therefore, to investigate the practical usefulness of non-invasive methods of monitoring exercise haemodynamics in this patient population., Methods: Using a novel impedance cardiography (ICG) approach that does not require basal impedance estimations and relies on a morphological analysis of the impedance signal (Signal-Morphology-ICG(™)), stroke volume (SV) and cardiac index (CI) were evaluated in 50 patients and 21 age-matched controls during a ramp-incremental cardiopulmonary exercise testing., Results: Technically unacceptable readings were found in 12 of 50 (24%) patients. In the remaining subjects, early decrease (N = 9) or a 'plateau' in SV (N = 8) and Δ (peak-unloaded exercise) SV <10 ml were markers of more advanced PAH (P<0.05). ΔCI ≤ 1.5-fold and early estimated lactate threshold were the only independent predictors of a severely reduced peak oxygen uptake (VO(2)) in patients (R(2) = 0.71, P<0.001). The finding of ΔCI ≤ 1.5-fold plus peak VO(2) < 50% predicted was associated with a number of clinical and functional markers of disease severity (P<0.001). In addition, abnormal SV responses and ΔCI ≤ 1.5-fold were significantly related to 1-year frequency of PAH-related adverse events (death and balloon atrial septostomy, N = 8; P<0.05)., Conclusions: 'Qualitative' and 'semi-quantitative' signal-morphology impedance cardiography(™) (PhysioFlow(™)) during incremental exercise provided clinically useful information to estimate disease severity and short-term prognosis in patients with PAH in whom acceptable impedance signals could be obtained., (© 2012 The Authors Clinical Physiology and Functional Imaging © 2012 Scandinavian Society of Clinical Physiology and Nuclear Medicine.)

Objective: To assess the immediate result of the modified Norwood procedure with new technique of anterograde regional cerebral perfusion (ARCeP) and retrograde regional coronary perfusion (RRCoP) in substitution of profound hypothermia and circulatory arrest in children with hypoplastic left heart syndrome (HLHS) with extremely hypoplastic ascending aorta (HA)., Methods: In the period of December of 2006 to February 2008 the modified Norwood procedure with tube between the right ventricle and pulmonary arteries or Sano shunt was performed in eight children with HLHS and with ascending aorta diameter less than 3 mm, (four male and four female) with median age of 9.2 days (ranging from 1 to 29 days) and median weigh of 3.3 kg (ranging from 2.7 to 3.8 kg). We used cardiopulmonary bypass and hypothermia at 25 masculineC with two venous cannulas and suture with a polytetrafluoroethylene graft sutured to the innominate artery as an arterial line and used to ARCeP. The RRCoP was performed by a deviation in the arterial line and placement of a catheter in the ascending aorta. The immediate surgical results and the clinical neurological manifestation were assessed., Results: The immediate surgical results revealed mortality of 25% and there were absence of neurological injury at clinical examination., Conclusions: The modified Norwood procedure can be accomplished with ARCeP and RRCoP in children with HLHS with HA with satisfactory immediate surgical result and without neurological complications.

Infracardiac total anomalous pulmonary venous drainage can be erroneously diagnosed as respiratory distress of several different etiologies during the neonatal period. A cross-sectional echocardiography study with Doppler color flow mapping can preoperatively determine the precise drainage site and pulmonary venous return pattern, thereby allowing cardiac surgery to be performed promptly, prior to any clinical deterioration.