Differential biventricular adaption to pulmonary vascular disease in patients with idiopathic/heritable and congenital heart disease: a prospective cardiac magnetic resonance and invasive study.

AIMS: Despite shared pathophysiological mechanisms, patients with idiopathic/heritable pulmonary arterial hypertension (IPAH/HPAH) have a poorer prognosis than those with PAH after congenital heart defect repair. Ventricular adaption remains unclear and could provide a basis for explaining differences in clinical outcomes. The aim of this prospective study was to assess clinical status, haemodynamic profile, and biventricular adaptation to PAH in children with various forms of PAH. METHODS AND RESULTS: Consecutive patients with IPAH/HPAH or post-operative PAH were prospectively recruited (n = 64). All patients underwent a comprehensive, protocolized assessment including functional assessment, measurement of brain natriuretic peptide (BNP) levels, invasive measurements, and a cardiac magnetic resonance (CMR) assessment. A cohort of age- and sex-matched healthy subjects served as controls. Patients with post-operative PAH had a better functional class (61.5 vs. 26.3% in Class I/II, P = 0.02) and a longer 6-min walk distance (320 ± 193 vs. 239 ± 156 m, P = 0.008) than IPAH/HPAH. While haemodynamic parameters were not significantly different between IPAH/HPAH and post-operative patients, post-operative patients with PAH presented with higher left ventricular volumes and better right ventricular function compared with patients with IPAH/HPAH (P < 0.05). On correlation analyses, left ventricular volumetric parameters were highly correlated with BNP and 6-min walk test distance in this population. CONCLUSION: Despite comparable haemodynamic profiles, patients with post-operative PAH had less functional limitation than their IPAH/HPAH counterparts. This is potentially related to the differential biventricular adaptation pattern evident on CMR with better myocardial contractility and higher left ventricular volumes in post-operative patients with PAH, highlighting the importance of ventriculo-ventricular interaction in the setting of PAH.

A 12-Year-Old Girl With Dyspnea and Unilateral Interlobular Septal Thickening.

CASE PRESENTATION: A 12-year-old girl was admitted to the hospital for exercise intolerance and radiographic abnormalities. She presented with a 5-year history of shortness of breath during intense exercise and did not undergo any medical evaluation. She felt that her symptoms had progressed with fever and right chest pain 2 months prior to admission. She was diagnosed with pneumonia by her chest CT scan, which revealed a small right hemithorax, interlobular septal thickening, and ground-glass opacity of the right lung (Fig 1A), and was treated with antibiotics. She soon became afebrile, but still felt shortness of breath during intense exercise. Then, a contrast-enhanced CT scan of the chest (not shown) showed the right lung still had interlobular septal thickening, and the right pulmonary artery was smaller than the left. There were no systemic symptoms, including skin rash and arthralgia. Family history showed her grandparents were cousins and her father had congenital dysarthria.

Additive value of 3T cardiovascular magnetic resonance coronary angiography for detecting coronary artery disease.

BACKGROUND: The purpose of the work was to evaluate the incremental diagnostic value of free-breathing, contrast-enhanced, whole-heart, 3 T cardiovascular magnetic resonance coronary angiography (CE-MRCA) to stress/rest myocardial perfusion imaging (MPI) and late gadolinium enhancement (LGE) imaging for detecting coronary artery disease (CAD). METHODS: Fifty-one patients with suspected CAD underwent a comprehensive cardiovascular magnetic resonance (CMR) examination (CE-MRCA, MPI, and LGE). The additive diagnostic value of MRCA to MPI and LGE was evaluated using invasive x-ray coronary angiography (XA) as the standard for defining functionally significant CAD (≥ 50% stenosis in vessels > 2 mm in diameter). RESULTS: 90.2% (46/51) patients (54.0 ± 11.5 years; 71.7% men) completed CE-MRCA successfully. On per-patient basis, compared to MPI/LGE alone or MPI alone, the addition of MRCA resulted in higher sensitivity (100% vs. 76.5%, p < 0.01), no change in specificity (58.3% vs. 66.7%, p = 0.6), and higher accuracy (89.1% vs 73.9%, p < 0.01) for CAD detection (prevalence = 73.9%). Compared to LGE alone, the addition of CE-MRCA resulted in higher sensitivity (97.1% vs. 41.2%, p < 0.01), inferior specificity (83.3% vs. 91.7%, p = 0.02), and higher diagnostic accuracy (93.5% vs. 54.3%, p < 0.01). CONCLUSION: The inclusion of successful free-breathing, whole-heart, 3 T CE-MRCA significantly improved the sensitivity and diagnostic accuracy as compared to MPI and LGE alone for CAD detection.

Assessment of right ventricular function with 320-slice volume cardiac CT: comparison with cardiac magnetic resonance imaging.

To evaluate the accuracy and feasibility of right ventricular function parameters measurement using 320-slice volume cardiac CT. Retrospective analysis of 50 consecutive patients (23 men, 27 women) with suspected pulmonary diseases was performed in electrocardiogram (ECG)-gated cardiac CT and cardiac magnetic resonance (CMR). Parameters including right ventricular end-diastolic volume (RVEDV), right ventricular end- systolic volume (RVESV), right ventricular stroke volume (RVSV), right ventricular cardiac output (RVCO), and right ventricular ejection fraction (RVEF) were semi-automatically and separately calculated from both CT and CMR data. Significant difference between measurements was measured by paired t test and two-variable linear regression analysis with Pearson's correlation coefficient. Bland-Altman analysis was performed in each pair of parameters. There was little variability between the measurements by the two observers (kappa = 0.895-0.980, P < 0.05). There was good correlation between all parameters obtained by CT and CMR (P < 0.001): RVEDV (108.5 ± 21.9 ml, 113.5 ± 24.8 ml, r = 0.944), RVESV (69.8 ± 33.4 ml, 73.2 ± 35.4 ml, r = 0.972), RVSV (39.0 ± 13.2 ml, 40.2 ± 13.3 ml, r = 0.977), RVCO (2.6 ± 0.7 l, 2.6 ± 0.7 l. r = 0.958), RVEF (38.8 ± 19.1 %, 39.1 ± 19.3 %, r = 0.990), and there was no significant difference between CT and CMR measurements in RVEF (n = 50, t = -0.677, P > 0.05). 320-slice volume cardiac CT is an accurate non-invasive technique to evaluate RV function.