Electrocardiographic Abnormalities and Their Association with Outcomes in Randomized Clinical Trials of Pulmonary Arterial Hypertension.

Rationale: Pulmonary arterial hypertension (PAH) is a progressive disease with manifestations including right atrial enlargement, right ventricular dysfunction, dilation, and hypertrophy. Electrocardiography (ECG) is a noninvasive, inexpensive test that is routinely performed in clinical settings. Prior studies have described separate abnormal findings in the electrocardiograms of patients with PAH. However, the role of composite ECG findings reflective of right heart disease (RHD) for risk stratification, clinical trial enrichment, and management of patients with PAH has not been explored. Objectives: To describe a pattern of RHD on ECG in patients with PAH and to investigate the association of this pattern with clinical measures of disease severity and outcomes. Methods: We harmonized individual participant data from 18 phase III randomized clinical trials of therapies for PAH (1998-2013) submitted to the U.S. Food and Drug Administration. RHD was defined as the presence of right ventricular hypertrophy, right axis deviation, right atrial enlargement, or right bundle branch block on ECG. Random effects linear regression, multilevel ordinal regression (cumulative link model), and Cox proportional hazards models were used to assess the association of RHD by ECG with 6-minute walk distance (6MWD), World Health Organization (WHO) functional class, and clinical worsening after a priori adjustment for age, sex, body mass index, and PAH etiology. Effect modification of treatment and ECG abnormalities was assessed by including an interaction term. Results: A total of 4,439 patients had baseline ECG, and 68% of patients had evidence of RHD. RHD on ECG was associated with higher pulmonary vascular resistance (P < 0.001) and higher mean pulmonary artery pressures (P < 0.001). Patients with RHD on ECG had 10 meters shorter 6MWD (P = 0.005) and worse WHO functional class (P < 0.001) at baseline. RHD on baseline ECG was associated with increased risk of clinical worsening (hazard ratio, 1.42; 95% confidence interval; 1.21, 1.67; P < 0.001). Patients with RHD had greater treatment effect in terms of 6MWD, WHO functional class, and time to clinical worsening than those without (P for interaction = 0.03, 0.001, and 0.03, respectively). Conclusions: RHD by ECG may be associated with worse outcomes and potentially greater treatment effect. Electrocardiograms could be an inexpensive, widely available noninvasive method to enrich clinical trial populations in PAH.

Implementation of a practice plan for the outpatient cardiac evaluation of children after acute SARS-CoV-2 infection and a report of outcomes.

SARS-CoV-2 infection has been associated with cardiovascular disease in children, but which children need cardiac evaluation is unclear. We describe our experience evaluating 206 children for cardiac disease following SARS-CoV-2 infection (one of whom had ventricular ectopy) and propose a new guideline for management of these children. Routine cardiac screening after SARS-CoV-2 infection in children without any cardiac signs or symptoms does not appear to be high yield.

Nanoparticle Delivery of STAT3 Alleviates Pulmonary Hypertension in a Mouse Model of Alveolar Capillary Dysplasia.

BACKGROUND: Pulmonary hypertension (PH) is a common complication in patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a severe congenital disorder associated with mutations in the FOXF1 gene. Although the loss of alveolar microvasculature causes PH in patients with ACDMPV, it is unknown whether increasing neonatal lung angiogenesis could prevent PH and right ventricular (RV) hypertrophy. METHODS: We used echocardiography, RV catheterization, immunostaining, and biochemical methods to examine lung and heart remodeling and RV output in Foxf1WT/S52F mice carrying the S52F Foxf1 mutation (identified in patients with ACDMPV). The ability of Foxf1WT/S52F mutant embryonic stem cells to differentiate into respiratory cell lineages in vivo was examined using blastocyst complementation. Intravascular delivery of nanoparticles with a nonintegrating Stat3 expression vector was used to improve neonatal pulmonary angiogenesis in Foxf1WT/S52F mice and determine its effects on PH and RV hypertrophy. RESULTS: Foxf1WT/S52F mice developed PH and RV hypertrophy after birth. The severity of PH in Foxf1WT/S52F mice directly correlated with mortality, low body weight, pulmonary artery muscularization, and increased collagen deposition in the lung tissue. Increased fibrotic remodeling was found in human ACDMPV lungs. Mouse embryonic stem cells carrying the S52F Foxf1 mutation were used to produce chimeras through blastocyst complementation and to demonstrate that Foxf1WT/S52F embryonic stem cells have a propensity to differentiate into pulmonary myofibroblasts. Intravascular delivery of nanoparticles carrying Stat3 cDNA protected Foxf1WT/S52F mice from RV hypertrophy and PH, improved survival, and decreased fibrotic lung remodeling. CONCLUSIONS: Nanoparticle therapies increasing neonatal pulmonary angiogenesis may be considered to prevent PH in ACDMPV.

Cardiopulmonary Testing before Pediatric Adenotonsillectomy for Severe and Very Severe Obstructive Sleep Apnea Syndrome.

OBJECTIVES/HYPOTHESIS: Adenotonsillectomy is first-line treatment for pediatric obstructive sleep apnea syndrome (OSAS) when not otherwise contraindicated. There is concern severe OSAS increases risk of comorbid cardiopulmonary abnormalities, such as ventricular hypertrophy or pulmonary hypertension, which preoperative testing could detect. Our objective is to determine if there is a severity of pediatric OSAS where previously undetected cardiopulmonary comorbidities are likely. STUDY DESIGN: Retrospective chart review. METHODS: We performed a retrospective review of 358 patients ≤21 years with severe OSAS who underwent adenotonsillectomy at a tertiary hospital June 1, 2016 to June 1, 2018. We extracted demographics, comorbidities, polysomnography, and preoperative tests. Wilcoxon rank-sum and logistic regression estimated associations of OSAS severity (based on obstructive apnea-hypopnea index [OAHI], hypoxia, hypercarbia) with preoperative echocardiograms and chest X-rays (CXRs). RESULTS: Mean age was 5.9 (±3.6) years and 52% were male. Mean OAHI and oxygen saturation nadir were 30.3 (±23.8) and 80.7% (±9.2), respectively. OAHI ≥60 was associated with having a preoperative echocardiogram (OR, 3.8; 95% CI, 1.7-8.5) or CXR (OR, 3.0; 95% CI, 1.4-6.8) compared to OAHI 10-59. There were no significant associations between OSAS severity and test abnormalities. The presence of previously diagnosed cardiopulmonary comorbidities was associated with abnormalities on echocardiogram (OR, 36; 95% CI, 4.1-320.1) and CXR (OR, 4.1; 95% CI, 1.2-14.4). CONCLUSIONS: Although pediatric patients with very severe OSAS (OAHI ≥60) underwent more pre-adenotonsillectomy cardiopulmonary tests, OSAS severity did not predict abnormal findings. Known cardiopulmonary comorbidities may be a better indication for cardiopulmonary testing than polysomnographic parameters, which could streamline pre-adenotonsillectomy evaluation and reduce cost. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2361-2368, 2021.

Impact of early sports specialisation on paediatric ECG.

Athletes of pediatric age are growing in number. They are subject to a number of risks, among them sudden cardiac death (SCD). This study aimed to characterize the pediatric athlete population in Switzerland, to evaluate electrocardiographic findings based on the International Criteria for electrocardiography (ECG) Interpretation in Athletes, and to analyze the association between demographic data, sport type, and ECG changes. Retrospective, observational study of pediatric athletes (less than 18 years old) including medical history, physical examination, and a 12-lead resting ECG. The primary focus was on identification of normal, borderline, and abnormal ECG findings. The secondary observation was the relation between ECG and demographic, anthropometric, sport-related, and clinical data. The 891 athletes (mean 14.8 years, 35% girls) practiced 45 different sports on three different levels, representing all types of static and dynamic composition of the Classification of Sports by Mitchell. There were 75.4% of normal ECG findings, among them most commonly early repolarization, sinus bradycardia, and left ventricular hypertrophy; 4.3% had a borderline finding; 2.1% were abnormal and required further investigations, without SCD-related diagnosis. While the normal ECG findings were related to sex, age, and endurance sports, no such observation was found for borderline or abnormal criteria. Our results in an entirely pediatric population of athletes demonstrate that sex, age, and type of sports correlate with normal ECG findings. Abnormal ECG findings in pediatric athletes are rare. The International Criteria for ECG Interpretation in Athletes are appropriate for this age group.

MicroRNA-21 regulates right ventricular remodeling secondary to pulmonary arterial pressure overload.

Right ventricular (RV) function is a critical determinant of survival in patients with pulmonary arterial hypertension (PAH). While miR-21 is known to associate with vascular remodeling in small animal models of PAH, its role in RV remodeling in large animal models has not been characterized. Herein, we investigated the role of miR-21 in RV dysfunction using a sheep model of PAH secondary to pulmonary arterial constriction (PAC). RV structural and functional remodeling were examined using ultrasound imaging. Our results showed that post PAC, RV strain significantly decreased at the basal region compared with t the control. Moreover, such dysfunction was accompanied by increases in miR-21 levels. To determine the role of miR-21 in RV remodeling secondary to PAC, we investigated the molecular alteration secondary to phenylephrine induced hypertrophy and miR21 overexpression in vitro using neonatal rat ventricular myocytes (NRVMs). We found that overexpression of miR-21 in the setting of hypertrophic stimulation augmented only the expression of proteins critical for mitosis but not cytokinesis. Strikingly, this molecular alteration was associated with an eccentric cellular hypertrophic phenotype similar to what we observed in vivo PAC animal model in sheep. Importantly, this hypertrophic change was diminished upon suppressing miR-21 in NRVMs. Collectively, our in vitro and in vivo data demonstrate that miR-21 is a critical contributor in the development of RV dysfunction and could represent a novel therapeutic target for PAH associated RV dysfunction.

Comparisons of traditional electrocardiographic criteria for left and right ventricular hypertrophy in young Asian women: The CHIEF heart study.

The performance of electrocardiographic (ECG) voltage criteria to identify left and right ventricular hypertrophy (LVH and RVH) in young Asian female adults have not been clarified so far.In a sample of 255 military young female adults, aged 25.2 years on average, echocardiographic LVH was respectively defined as the left ventricular mass (LVM) indexed by body surface area (BSA) (≥88 g/m) and by height (≥41 g/m), and RVH was defined as anterior right ventricular wall thickness >5.2 mm. The performance of ECG voltage criteria for the echocardiographic LVH and RVH were assessed by area under curve (AUC) of receiver operating characteristic (ROC) curve to estimate sensitivity and specificity.For the Sokolow-Lyon (the maximum of SV1 or SV2 + RV5 or RV6) and Cornell (RaVL + SV3) voltage criteria with the LVM/BSA ≥88 g/m, the AUC of ROC curves were 0.66 (95% confidence intervals [CI]: 0.52-0.81, P = .039) and 0.61 (95% CI: 0.44-0.77, P = .18), respectively. For these 2 ECG voltage criteria with the LVM/height ≥41 g/m, the AUC of ROC curves were 0.64 (95% CI: 0.52-0.75, P = 0.11) and 0.73 (95% CI: 0.61-0.85, P = 0.0074), respectively. The best cut-off points selected for the Sokolow-Lyon and Cornell voltage criteria with echocardiographic LVH in young Asian females were 26 mm and 6 mm, respectively. In contrast, all the AUC of ROC curves were less than 0.60 and not significant according to the Sokolow-Lyon (the maximum of RV1 + SV5 or V6) and Myers' voltage criteria (eg, the voltage of R wave in V1 and the ratios of R/S in V1, V5 and V6) with echocardiographic RVH.There was a suggestion that the ECG voltage criteria to screen the presence of LVH should be adjusted for the young Asian female adults, and with regard to RVH, the ECG voltage criteria were found ineffective.

Management of Unoperated Tetralogy of Fallot in a 59-Year-Old Patient.

Tetralogy of Fallot is the most common cyanotic congenital heart defect consisting of an overriding aorta, right ventricular outflow obstruction, ventricular septal defect, and right ventricular hypertrophy. Without surgical management, approximately only 3% of patients survive past the age of 40 years. Cases of unoperated patients reaching adulthood have been reported; however, few studies describe treatment guidelines for surgical or therapeutic management. In this article, we report the case of a 59-year-old Hispanic male with unoperated tetralogy of Fallot presenting to our cardiology clinic for initial workup and management.

An easy method for monitoring patients with pulmonary Hypertension: P-wave dispersion.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a haemodynamic and pathophysiological condition with restricted flow through the pulmonary arterial circulation. In pulmonary hypertension, right ventricular hypertrophy and diastolic dysfunction can lead to an increase in atrial strain, fibrosis and dilation, which cause inhomogeneous atrial conduction. Interlead variation in P-wave duration is called P-wave dispersion (PwD), which is an electrocardiographic parameter that can be used to predict atrial arrhythmias. Our aim was to investigate the relationship between PwD, functional capacity, and invasive and non-invasive haemodynamic parameters of patients diagnosed with PAH. METHODS: Between 2015 and 2017 we enrolled 33 patients admitted to our in-patient clinic and diagnosed with PAH, and 32 healthy individuals for the control group. Details of these patients at the time of diagnosis were analysed, including gender, age, physical examination, electrocardiogram (ECG), echocardiography, six-minute walk test distance (6MWD), haemodynamic parameters and blood tests for biochemical markers that are correlated with clinical severity. Statistical analyses were performed using SPSS version 20.0 (SPSS Inc, Chicago, Illinois, USA). Statistical significance was taken as p < 0.05. RESULTS: In the forward stepwise multiple linear regression analysis, PwD and mean pulmonary artery pressure determined by right heart catheterisation were independently related to the functional capacity tested by the 6MWD (p < 0.02 and p < 0.01, respectively). CONCLUSIONS: PwD can easily be calculated from a surface ECG to indirectly estimate the functional status and prognosis of the patient with PAH.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

The evolution of electrocardiographic signs of right ventricular overload after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Balloon pulmonary angioplasty (BPA) is a treatment option for a patient with chronic thromboembolic pulmonary hypertension. OBJECTIVES: We aimed to investigate the evolution of electrocardiographic (ECG) markers of right ventricular hypertrophy (RVH) after BPA. PATIENTS AND METHODS: Standard 12­lead ECG was performed in 41 patients with chronic thromboembolic pulmonary hypertension before the first BPA and after completion of treatment. RESULTS: In the whole study group, the percentage change in the pulmonary vascular resistance (PVR) after BPA correlated with the percentage change in the values of the following ECG parameters (P <0.05): axis of the QRS (rho = 0.530) and T wave (rho = 0.372); P wave in leads II (rho = 0.340) and III (rho = 0.430); S wave in lead V5 (rho = 0.634); R/S ratio in lead V5 (rho = -0.636); S wave in lead V6 (rho = 0.508); S wave in lead I (rho = 0.496). Then, the group was divided into 2 subgroups: group A, defined as a drop in PVR greater than the median value (49%) for the whole study population; and group B, defined as a drop in PVR below the median value. In group A, the following parameters changed after BPA: T­wave axis (P <0.001), P wave in lead II (P <0.001), S wave in lead V5 (P <0.001), R/S ratio in lead V5 (P <0.001). In group B, despite some hemodynamic and functional improvement, there were no differences in ECG mark ers of RVH after BPA. CONCLUSIONS: An improvement in RVH parameters can be observed on ECG after a hemodynamically effective BPA.

Respuesta al ECG de mayo de 2019 / Response to ECG, May 2019

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Right Ventricular Function After Pulmonary Artery Banding: Adaptive Processes Assessed by CMR and Conductance Catheter Measurements in Sheep.

This experimental study describes the adaptive processes of the right ventricular (RV) myocardium after pulmonary artery banding (PAB) evaluated by cine cardiac magnetic resonance (CMR), phase-contrast CMR (PC-CMR), and conductance catheter. Seven sheep were subjected to CMR 3 months after PAB. Conductance catheter measurements were performed before and 3 months after PAB. Four nonoperated, healthy, age-matched animals served as controls. Higher RV masses (p < 0.01), elevated RV end-systolic volumes (p < 0.05), and lower RV ejection fraction (p < 0.01) were observed in the operated group. The time-to-peak pulmonary artery flow was longer in the banding group (p < 0.01). RV maximal pressure and RV end-diastolic pressure correlated with the time-to-peak flow in the pulmonary artery (r = - 0.70 and - 0.69, respectively). In summary, PAB caused RV hypertrophy, increased myocardial contractility, and decreased RV-EF and cardiac output. The time-to-peak pulmonary artery flow correlated with RV pressures.

Physicians' Ability to Visually Estimate Left Ventricular Ejection Fraction, Right Ventricular Enlargement, and Paradoxical Septal Motion After a 2-Day Focused Cardiac Ultrasound Training Course.

OBJECTIVE: Focused cardiac ultrasound (FCU) can provide useful information for the management of shock and acute respiratory distress syndrome. This study aimed to determine whether a 2-day focused cardiac ultrasound training course could enable critical care physicians to interpret ultrasound images in terms of left ventricular ejection fraction (LVEF), ratio of right ventricular end-diastolic area to left ventricular end-diastolic area (R/LVEDA), and septal kinetics. DESIGN: A prospective analysis of an image test score. SETTING: Ultrasound training programs in 7 regions across China. PARTICIPANTS: Two hundred forty-seven critical care physicians. INTERVENTIONS: All participants received a 2-day FCU training, including 4 sessions of basic heart function appraisal, 3 sessions of hands-on practice, and 1 session of image interpretation. MEASUREMENTS AND MAIN RESULTS: The post-training total scores were considerably higher than those of pretraining (75.6% v 58.9%, respectively, p < 0.001). After the course, the trainees obtained considerably higher scores on images with LVEF <30% than on images with LVEF 30% to 54% and LVEF ≥55% (100% v 60.0% and 60.0%, respectively, p < 0.001). The trainees obtained considerably higher scores on images with R/LVEDA >1 than on images with R/LVEDA 0.6 to 1 and R/LVEDA <0.6 (90.0% v 80.0% and 80.0%, p = 0.042 and p < 0.001, respectively). The trainees obtained considerably higher scores on images with paradoxical septal movement (PSM) than on images without PSM (100% v 75.0%, respectively, p < 0.001). CONCLUSION: The physicians' abilities to assess LVEF, RV enlargement, and PSM improved after the training course, and they demonstrated more accurate estimations of the most obviously abnormal images.

A Pulmonary Trunk Banding Model of Pressure Overload Induced Right Ventricular Hypertrophy and Failure.

Right ventricular (RV) failure induced by sustained pressure overload is a major contributor to morbidity and mortality in several cardiopulmonary disorders. Reliable and reproducible animal models of RV failure are therefore warranted in order to investigate disease mechanisms and effects of potential therapeutic strategies. Banding of the pulmonary trunk is a common method to induce isolated RV hypertrophy but in general, previously described models have not succeeded in creating a stable model of RV hypertrophy and failure. We present a rat model of pressure overload induced RV hypertrophy caused by pulmonary trunk banding (PTB) that enables different phenotypes of RV hypertrophy with and without RV failure. We use a modified ligating clip applier to compress a titanium clip around the pulmonary trunk to a pre-set inner diameter. We use different clip diameters to induce different stages of disease progression from mild RV hypertrophy to decompensated RV failure. RV hypertrophy develops consistently in rats subjected to the PTB procedure and depending on the diameter of the applied banding clip, we can accurately reproduce different disease severities ranging from compensated hypertrophy to severe decompensated RV failure with extra-cardiac manifestations. The presented PTB model is a valid and robust model of pressure overload induced RV hypertrophy and failure that has several advantages to other banding models including high reproducibility and the possibility of inducing severe and decompensated RV failure.

Utility of the amplitude of RV1+SV5/6 in assessment of pulmonary hypertension.

Electrocardiogram (ECG) has been widely used for assessment of right ventricular (RV) hypertrophy (RVH) in patients with pulmonary hypertension (PH). However, it still remains unclear which ECG criteria of RVH are useful to predict for the severity of PH. The aim of our study was to examine the utility of ECG findings of RVH in assessment of PH. A total of 53 patients (42 women, mean age; 57.6 ± 16.4 years) with pre-capillary PH, who were diagnosed by right heart catheterization, underwent blood sampling, ECG, and cardiac magnetic resonance within a week before the right heart catheterization. We assessed the traditional ECG criteria of RVH in PH patients, and compared to age- and gender-matched control subjects without PH confirmed by 2-dimensional echocardiography (n = 42, mean age 55.3 ± 15.9 years). We also analyzed the clinical variables associated with ECG findings in patients with PH. Mean pulmonary arterial pressure (mPAP), cardiac index, and pulmonary vascular resistance (PVR) in PH patients were 35.3 ± 11.9 mmHg, 2.82 (2.09-3.45) L/min/m2, and 576 ± 376 dyne·sec·cm-5, respectively. The prevalence of right axis deviation (43.4%), R:S ratio V1 > 1 (32.1%), and RV1+SV5/6 > 10.5 mm (69.8%) in PH patients was greater than those in control subjects (p < 0.001). In univariate analysis, mPAP, PVR, RV wall thickness, RV mass index, RV volume, and RV ejection fraction (EF) (inversely) were significantly correlated with the amplitude of RV1+SV5/6. Multiple regression analysis revealed that mPAP and RVEF (inversely) were independently associated with the amplitude of RV1+SV5/6 (R2 = 0.282). Also, we performed the survival analysis among pre-capillary PH patients. During a mean follow-up of 3.7 years, patients with ≥ 16.4 mm of RV1+SV5/6 had worse prognosis than those with < 16.4 mm (Log rank p = 0.015). In conclusion, the amplitude of SV1+RV5/6 could be the most useful factor reflected for RV remodeling, hemodynamics and survival in patients with pre-capillary PH.

Right Ventricular Hypertrophy and Dilation in Patients With Human Immunodeficiency Virus in the Absence of Clinical or Echocardiographic Pulmonary Hypertension.

BACKGROUND: Involvement of right-sided heart chambers (RSHCs) in patients infected with human immunodeficiency virus (HIV) is common and is usually attributed to pulmonary arterial or venous hypertension (PH). However, myocardial involvement in patients with HIV is also common and might affect RSHCs even in the absence of overt PH. Our aim was to define morphologic and functional alterations in RSHC in patients with HIV and without PH. METHODS AND RESULTS: A total of 50 asymptomatic patients with HIV and 25 control subjects without clinical or echocardiographic signs for PH were included in the study. Transthoracic echocardiography was used to obtain measurements. Patients with HIV had significantly increased right ventricular end-diastolic diameter (RVEDD) and right ventricular free wall thickness (RVFWT), as well as increased right atrial area and pulmonary arterial diameter, compared with control subjects. After adjustment for age, sex, and body surface area, RVFWT (average 1.81 mm, 95% confidence interval [CI] 0.35-3.26 mm) and RVEDD (average 6.82 mm, 95% CI 2.40-11.24 mm) were significantly higher in subjects infected with HIV. More patients with right ventricular hypertrophy were on antiretroviral treatment, and RVFWT was on average 1.3 mm higher (95% CI 0.24-2.37 mm) in patients on antiretroviral treatment after adjustment for confounders. CONCLUSIONS: These findings suggest that alterations in RSHCs were present in patients with HIV without PH.

Eisenmenger syndrome - an electrocardiographic and echocardiographic assessment of the right ventricle.

BACKGROUND: Eisenmenger syndrome represents severe, irreversible, and end-stage pulmonary arterial hypertension (PAH) associated with congenital heart defects. For long-term outcome optimal right ventricular (RV) adaptation is crucial with precise assessment of its hypertrophy, dilatation and function. OBJECTIVES: Associations of electrocardiographic (ECG) and echocardiographic (ECHO) RV characteristics were analyzed. METHODS: Included were 52 patients (39F/13M), median age 45 years (24-78). Following ECG parameters were analyzed: Butler-Leggett formula (B-L), Sokolow-Lyon criterion (S-L), QRS duration (QRS), maximum spatial QRS vector magnitude (QRS max); and ECHO parameters: RV diameter (RVd), RV wall thickness (RVAW), RV/LV function. RESULTS: Following significant ECG-ECHO associations were demonstrated: S-L criterion and B-L formula with RVAW (p 120 ms only with severely dilated RV (RVd > 45 mm), while QRS max 33 mm); A new combined scoring system was introduced. CONCLUSIONS: In Eisenmenger syndrome RV hypertrophy is compensatory; diagnosis of prognostically unfavorable RV dilatation is therefore important. Combined ECG-ECHO analysis enables more accurate risk stratification. QRS duration > 120 ms seems to be a late marker; QRS max together with ECHO parameters may help to distinguish patients at higher risk for clinical deterioration (Tab. 3, Fig. 8, Ref. 53).

Severe right ventricular hypertrophy in a patient with extracardiac and intracardiac shunt.

Cardiac MRI is a complementary and confirmatory modality to a clinical echocardiography in diagnosing patients with complex adult congenital heart disease, especially in presence of great vessel abnormalities. We present a unique case of a patient with pulmonary hypertension (PH), severe right ventricular hypertrophy, Gerbode defect, and a large patent ductus arteriosus (PDA). The diagnosis of PDA was not visualized on prior serial echocardiograms and discovered on a comprehensive cardiac MRI/Chest MR angiogram.