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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.

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Vitamin K antagonists are advised in pulmonary arterial hypertension patients despite a lack of safety data. We reviewed major bleeding in three classes of pulmonary hypertension patients, all receiving vitamin K antagonists. Bleeding event rates were 5.4 per 100 patient-years for patients with idiopathic pulmonary arterial hypertension, 19 per 100 patient-years for connective tissue disease related pulmonary arterial hypertension patients and 2.4 per 100 patient-years for chronic thromboembolic pulmonary hypertension patients. Life tables analysis showed that event-free survival was worse in patients with connective tissue disease related pulmonary hypertension than in patients with idiopathic pulmonary arterial hypertension (Wilcoxon=12.8; p<0.001), and patients with chronic thromboembolic pulmonary hypertension (Wilcoxon=23.2; p<0.001). Patients with idiopathic pulmonary arterial hypertension suffered more events than patients with chronic thromboembolic pulmonary hypertension (Wilcoxon=7.2; p<0.01). Major bleeding was independent of age, sex, target international normalised ratio (INR) range, documented INR, vitamin K antagonist type, or right atrial pressure, but was associated with use of prostacyclin analogues. Major bleeding risk during vitamin K antagonist therapy differs among groups of patients with pulmonary hypertension. Further research regarding optimal anticoagulant therapy is needed, as well as risk-benefit analyses for pulmonary hypertension patients with a higher bleeding propensity.

A 62-year-old man with a history of significant alcohol consumption presented with atypical neurological symptoms. Bacterial meningitis, caused by Streptococcus pneumoniae, was diagnosed with a 24-hour delay. Despite antibiotic treatment, the patient developed heart failure and multiple organ failure. He also had endocarditis with insufficient mitral and aortic valves. Subsequently, we found signs of a pulmonary infection. Because of an unsustainable haemodynamic situation, double heart valve replacement was considered necessary, despite the extremely high surgical risk and the extracardial infection foci showed by leukocyte scintigraphy. The patient died shortly after surgery. The triad of meningitis, pneumonia and endocarditis caused by Streptococcus pneumoniae is called the Austrian syndrome. This syndrome is rare and often has a serious course. It is strongly associated with asplenia, functional asplenia or hyposplenism, as occurs with alcohol abuse. Early recognition and a combination of antibiotic and surgical treatment is essential.

Heart rate (HR) at rest is an important marker of prognosis in heart failure, but has not been addressed in pulmonary arterial hypertension (PAH). To determine the prognostic value of HR at rest in patients with PAH, we retrospectively analyzed 140 consecutive patients with idiopathic PAH. Electrocardiogram (ECG)-derived HR at rest was evaluated as a potential predictor of adverse prognosis (death or lung transplantation), in addition to World Health Organization functional class, 6-minute walk distance, and hemodynamics before and approximately 1 year and 2 years after initiation of PAH treatment. During follow-up, 49 patients (35%) died, and 5 patients (4%) underwent lung transplantation. Before treatment initiation and after 1 year and 2 years of treatment, respectively, a higher HR at rest was an independent predictor of adverse prognosis (hazard ratios per 10-beats/min increase 1.76, 95% confidence interval 1.42 to 2.18, 2.31, 95% confidence interval 1.58 to 3.38, 2.1, 95% confidence interval 1.39 to 3.19, respectively, p <0.001 for all). Change in HR between the first and last ECG also independently predicted prognosis (hazard ratio per 1-beat/min increase 1.03, 95% confidence interval 1.01 to 1.06). In conclusion, a higher HR at rest and an important increase in HR at rest during follow-up signify a considerable risk of death in patients with PAH. ECG-derived HR at rest is an important marker of prognosis and should be assessed before and at frequent intervals after initiation of treatment for PAH.

Background: The potential use of the ECG for monitoring treatment effects in patients with pulmonary arterial hypertension (PAH) has not been investigated. We evaluated whether the ECG is useful for monitoring treatment response based on changes in pulmonary vascular resistance (PVR)., Methods: An ECG was recorded in 81 PAH patients at the time of diagnostic right heart catheterization and after 1 year of treatment. Patients were treated according to the guidelines. Patients were divided into two groups based on PVR (ie, < 500 or > 500 dyne x s x cm(-5)). A positive treatment response was defined as a > 25% decrease in PVR to an absolute PVR of < 500 dyne x s x cm(-5)., Results: At baseline, the 19 patients with a PVR of < 500 dyne x s x cm(-5) had a significantly lower P amplitude in lead II, a less rightward oriented QRS axis, and a more rightward T axis than the 62 patients with a PVR of > 500 dyne x s x cm(-5). Overall (n = 81), the mean (+/- SD) change in PVR was -143 +/- 360 dyne x s x cm(-5) after 1 year of treatment (p < 0.001). Twelve patients (19%) with a baseline PVR of > 500 dyne x s x cm(-5) were classified as responders. Receiver operating characteristic analysis determined that the P amplitude in lead II (area under the curve [AUC], 0.80; 95% confidence interval [CI], 0.67 to 0.94; p < 0.01), QRS axis (AUC, 0.70; 95% CI, 0.52 to 0.89; p = 0.03), and T axis (AUC, 0.90; 95% CI, 0.82 to 0.97; p < 0.001) were important determinants of treatment response. The presence of a P amplitude in lead II of < 0.175 mV and a T axis of >or= 25 degrees combined had a positive and negative predictive value for treatment response of 0.81 (95% CI, 0.37 to 0.96) and 0.94 (95% CI, 0.86 to 0.99), respectively., Conclusions: Routine ECG evaluation can be an important contribution in the assessment of treatment response in PAH patients.

Background and Purpose: Normal limits of the spatial QRS-T angle and spatial ventricular gradient (SVG) are only available from Frank vectorcardiograms (VCGs) of male subjects. We determined normal limits for these variables derived from standard 12-lead electrocardiograms (ECGs) of 660 male and female students aged 18 to 29 years., Methods: A computer algorithm was used that constructed approximated VCG leads by inverse Dower matrix transformation of the 12-lead ECG and subsequently calculated the spatial QRS-T angle, SVG magnitude, and orientation., Results: In female subjects, the QRS-T angle was more acute (females, 66 degrees +/- 23 degrees; normal, 20 degrees-116 degrees; males, 80 degrees +/- 24 degrees; normal, 30 degrees-130 degrees; P < .001), and the SVG magnitude was smaller (females, 81 +/- 23 mV x ms; normal, 39-143 mV x ms; males, 110 +/- 29 mV x ms; normal, 59-187 mV x ms; P < .001) than in male subjects. The male SVG magnitude in our study was larger than that computed in Frank VCGs (79 +/- 28 mV.ms; P < .001)., Conclusions: The spatial QRS-T angle and SVG depend strongly on sex. Furthermore, normal limits of SVG derived from Frank VCGs differ markedly from those derived from VCGs synthesized from the standard ECG. As nowadays, VCGs are usually synthesized from the 12-lead ECG; normal limits derived from the standard ECG should preferably be used.

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.).

The study aimed to assess whether the 12-lead ECG-derived ventricular gradient, a vectorial representation of ventricular action potential duration heterogeneity directed toward the area of shortest action potential duration, can improve ECG diagnosis of chronic right ventricular (RV) pressure load. ECGs from 72 pulmonary arterial hypertension patients recorded <30 days before onset of therapy were compared with ECGs from matched healthy control subjects (n = 144). Conventional ECG criteria for increased RV pressure load were compared with the ventricular gradient. In 38 patients a cardiac magnetic resonance (CMR) study had been performed within 24 h of the ECG. By multivariable analysis, combined use of conventional ECG parameters (rsr' or rsR' in V1, R/S > 1 with R > 0.5 mV in V1, and QRS axis >90 degrees ) had a sensitivity of 89% and a specificity of 93% for presence of chronic RV pressure load. However, the ventricular gradient not only had a higher diagnostic accuracy for chronic RV pressure load by receiver operating characteristic analysis [areas under the curve (AUC) = 0.993, SE 0.004 vs. AUC = 0.945, SE 0.021, P < 0.05], but also discriminated between mild-to-moderate and severe RV pressure load. CMR identified an inverse relation between the ventricular gradient and RV mass, and a trend toward a similar relation with RV volume. In conclusion, chronically increased RV pressure load is electrocardiographically reflected by an altered ventricular gradient associated with RV remodeling-related changes in ventricular action potential duration heterogeneity. The use of the ventricular gradient allows ECG detection of even mildly increased RV pressure load.

Objective: To assess the effect of pulmonary valve replacement (PVR) on the repolarization of patients with tetralogy of Fallot., Background: Pulmonary valve regurgitation may cause right ventricular failure in adult patients with Fallot's tetralogy. In these patients, prolonged depolarization and disturbed repolarization are associated with ventricular arrhythmias and sudden cardiac death., Methods: Thirty Fallot patients (age 32+/-9 years, 19 male) eligible for PVR were studied with cardiac magnetic resonance imaging (CMR) before and 6 months after PVR. Electrocardiograms obtained during initial and follow-up CMR were analyzed and occurrence of ventricular arrhythmias was studied., Results: Right ventricular end-diastolic volume (RV EDV) decreased from 322+/-87 to 215+/-57 ml after PVR (P<0.0001). The spatial QRS-T angle normalized from 117+/-34 to 100+/-35 degrees , P=0.0004 (normal angle <105 degrees). QT dispersion and T-wave complexity did not change significantly. T-wave amplitude decreased from 376+/-121 to 329+/-100 microV (P=0.01). T-wave area decreased from 43+/-15 to 38+/-13 microV s (P=0.02). Decreases in T-wave amplitude and area were most prominent in the right precordial leads overlying the RV. Three patients had sustained ventricular arrhythmias and one patient died suddenly. These patients had a QRS duration >160 ms. No severe ventricular arrhythmias were found in patients with a RV EDV <220 ml, QRS-T angle <100 degrees , QT dispersion <60 ms or T-wave complexity <0.30., Conclusion: Normal repolarization indices may be associated with the absence of severe ventricular arrhythmias. PVR in Fallot patients with dilated right ventricles has a beneficial effect on electrocardiographic indices of repolarization heterogeneity.

Aims: This study investigates whether increased right ventricular (RV) pressure in pulmonary hypertension (PH) impairs right coronary artery (RCA) flow and RV perfusion., Methods: In 25 subjects, five patients with idiopathic pulmonary arterial hypertension, nine patients with chronic thromboembolic pulmonary arterial hypertension, and 11 healthy controls, flow of the RCA and left anterior descending (LAD) artery was measured with MR flow quantification., Results: In PH, RCA peak systolic and mean systolic flow were lower, 1.02 +/- 0.62 mL/s and 0.42 +/- 0.30 mL/s, than peak and mean diastolic flow, 2.99 +/- 1.97 mL/s (P < 0.001) and 1.73 +/- 0.97 mL/s (P < 0.001); a pattern similar to the LAD. In contrast, in controls, RCA peak and mean flow in systole, 1.63 +/- 0.58 mL/s and 0.72 +/- 0.23 mL/s, were comparable to peak and mean flow in diastole, 1.72 +/- 0.48 mL/s and 0.93 +/- 0.28 mL/s (NS). The systolic-to-diastolic flow ratio in the RCA, and mean flow per gram RV tissue, were inversely related to RV mass, R = -0.61 (P = 0.009), and R = -0.73 (P < 0.001) and to RV pressure, R = -0.83 (P < 0.001), and R = -0.57 (P = 0.033)., Conclusion: Although in controls, RCA flow is similar in systole and diastole, in PH there is systolic flow impediment, which is proportional to RV pressure and mass. In patients with severe RV hypertrophy total mean flow is reduced.

The study aim was to assess three-dimensional electrocardiogram (ECG) changes during development of pulmonary arterial hypertension (PAH). PAH was induced in male Wistar rats (n = 23) using monocrotaline (MCT; 40 mg/kg sc). Untreated healthy rats served as controls (n = 5). ECGs were recorded with an orthogonal three-lead system on days 0, 14, and 25 and analyzed with dedicated computer software. In addition, left ventricular (LV)-to-right ventricular (RV) fractional shortening ratio was determined using echocardiography. Invasively measured RV systolic pressure was 49 (SD 10) mmHg on day 14 and 64 (SD 10) mmHg on day 25 vs. 25 (SD 2) mmHg in controls (both P < 0.001). Baseline ECGs of controls and MCT rats were similar, and ECGs of controls did not change over time. In MCT rats, ECG changes were already present on day 14 but more explicit on day 25: increased RV electromotive forces decreased mean QRS-vector magnitude and changed QRS-axis orientation. Important changes in action potential duration distribution and repolarization sequence were reflected by a decreased spatial ventricular gradient magnitude and increased QRS-T spatial angle. On day 25, LV-to-RV fractional shortening ratio was increased, and RV hypertrophy was found, but not on day 14. In conclusion, developing PAH is characterized by early ECG changes preceding RV hypertrophy, whereas severe PAH is marked by profound ECG changes associated with anatomical and functional changes in the RV. Three-dimensional ECG analysis appears to be very sensitive to early changes in RV afterload.

Background: Predicting changes in right ventricular (RV) size and function after pulmonary valve replacement (PVR) is important for timely reintervention in adult tetralogy of Fallot patients., Methods: We analyzed the influence of pulmonary regurgitation severity and RV size and function before PVR on the outcome of RV size and function after PVR in 27 adult Fallot patients who had cardiac magnetic resonance imaging before and after PVR. RV dimensions were indexed for body surface area., Results: Pulmonary regurgitation (48% +/- 11% of RV stroke volume) was not related to RV dimensions and function before PVR. Moreover, severity of pulmonary regurgitation did not influence changes in RV dimensions after PVR. The indexed RV end-systolic volume before PVR (mean, 98 mL/m2; range, 52 to 235 mL/m2) best predicted the indexed RV end-systolic volume after PVR (mean, 59 mL/m2; range, 24 to 132 mL/m2, r = 0.78, p < 0.001) and the indexed RV end-diastolic volume after PVR (mean, 107 mL/m2; range, 70 to 170 mL/m2, r = 0.73, p < 0.001). Baseline RV ejection fraction corrected for valvular insufficiencies and shunting (21% +/- 7%) best predicted the RV ejection fraction after PVR (43% +/- 10%, r = 0.77, p < 0.001)., Conclusions: Timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction rather than on severity of pulmonary regurgitation.