CMR assessment of right ventricular function in patients with combined pulmonary stenosis and insufficiency after correction of tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is one of the most common types of congenital heart disease and requires prompt surgical correction. Post-correction pulmonary insufficiency (PI) often ensues in adulthood. At times, the PI is accompanied by residual pulmonary stenosis (PS). Little is known regarding right ventricular (RV) function in the setting of combined PS and PI. PURPOSE: To compare cardiac magnetic resonance (CMR) parameters for the assessment of RV function between patients with combined pulmonary stenosis and pulmonary insufficiency (PSPI) and isolated PI following surgical repair of TOF. MATERIAL AND METHODS: Retrospective review of patients with comparable corrected TOF and similar PI was performed. Seventeen patients (median age, 24 years; range, 10-52 years) had combined PSPI and 30 patients (median age, 30 years; range, 6-70 years) had isolated PI. Cine magnetic resonance (MR) images (Philips Medical Systems, Best, The Netherlands) in the short-axis plane were used to calculate end-systolic, end-diastolic, and stroke volumes (RVESV, RVEDV, RVSV) and to measure RV wall thickness. Velocity-encoded cine MR images were used to measure pulmonary regurgitation fraction (PRF) by calculating the ratio of backward flow and total forward flow, obtained from the main pulmonary flow analysis. Peak pressure gradient across the pulmonary valve was obtained from spectral Doppler echocardiography. RESULTS: RVEF was 51 ± 8% in the PSPI patients and 39 ± 11%, in the patients with isolated PI (P = 0.001). Additionally, RV wall thickness was 5.2 ± 0.8 mm in the PSPI patients compared to 2.6 ± 0.9 mm in the isolated PI patients (P = 0.001). RVESVi and RVEDVi were significantly lower (P < 0.05) in patients with combined PSPI (60 ± 21 mL/m(2), 121 ± 35 mL/m(2), respectively) compared to the patients with isolated PI (95 ± 48 mL/m(2), 152 ± 61 mL/m(2), respectively). CONCLUSION: RV function is preserved in patients with PSPI when compared to patients with PI following surgical repair of TOF.

Collateral flow measurement by phase-contrast magnetic resonance imaging for the assessment of systemic venous baffle patency after atrial switch repair for transposition of the great arteries.

PURPOSE: We aimed to describe and compare azygos vein flow patterns of patients with obstructed and unobstructed systemic venous baffle after atrial switch repair for d-transposition of the great arteries (TGA). We hypothesized that phase-contrast magnetic resonance imaging would enable characterization of retrograde collateral flow across the azygos vein in cases of systemic venous baffle obstruction. MATERIALS AND METHODS: This is a retrospective, cross-sectional study. Twelve patients with atrial switch repair for TGA were examined. Azygos flow index was measured with phase-contrast magnetic resonance imaging; comparison was made between patients with documented systemic venous baffle obstruction (n=3) and a control group of patients without baffle obstruction (n=9). RESULTS: Patients with systemic venous baffle obstruction had a distinctive azygos flow pattern that was retrograde and an increased amount of azygos flow compared with patients without obstruction [median (range), -436 (-455/-399) vs. 103 (51/125) mL/min/m2; P=0.01]. CONCLUSIONS: Patients with systemic venous baffle obstruction have a characteristic collateral flow across the azygos vein. Azygos vein flow measurement may be used for the assessment of baffle patency in patients after atrial switch repair for TGA. However, diagnostic accuracy needs to be tested in a larger population.

Usefulness of cardiovascular magnetic resonance imaging to predict the need for intervention in patients with coarctation of the aorta.

Cardiovascular magnetic resonance (CMR) imaging can predict hemodynamically significant coarctation of the aorta (CoA) with a high degree of discrimination. However, the ability of CMR to predict important clinical outcomes in this patient population is unknown. Therefore, we sought to define the ability of CMR to predict the need for surgical or transcatheter intervention in patients with CoA. We retrospectively reviewed the data from 133 consecutive patients who had undergone CMR for the evaluation of known or suspected CoA. The characteristics of the CMR-derived variables predicting the need for surgical or transcatheter intervention for CoA within 1 year were determined through logistic regression analysis. Therapeutic aortic intervention was performed in 41 (31%) of the 133 patients during the study period. The indexed minimum aortic cross-sectional area was the strongest predictor of subsequent intervention (area under the receiver operating characteristic curve 0.975) followed by heart rate-corrected deceleration time in the descending aorta (area under the receiver operating characteristic curve 0.951), and the percentage of flow increase (area under the receiver operating characteristic curve 0.867). The combination of the indexed minimum aortic cross-sectional area and rate-corrected deceleration time in the descending aorta provided the best predictive model (area under the receiver operating characteristic curve 0.986). In conclusion, CMR findings can predict the need for subsequent intervention in CoA. These findings reinforce the "gate-keeper role" of CMR to cardiac catheterization by providing valuable diagnostic and powerful prognostic information and could guide additional treatment of patients with CoA with the final intent of reducing the number of diagnostic catheterizations in such patients.

Prediction of hemodynamic severity of coarctation by magnetic resonance imaging.

A published formula containing minimal aortic cross-sectional area and the flow deceleration pattern in the descending aorta obtained by cardiovascular magnetic resonance predicts significant coarctation of the aorta (CoA). However, the existing formula is complicated to use in clinical practice and has not been externally validated. Consequently, its clinical utility has been limited. The aim of this study was to derive a simple and clinically practical algorithm to predict severe CoA from data obtained by cardiovascular magnetic resonance. Seventy-nine consecutive patients who underwent cardiovascular magnetic resonance and cardiac catheterization for the evaluation of native or recurrent CoA at Children's Hospital Boston (n = 30) and the University of California, San Francisco (n = 49), were retrospectively reviewed. The published formula derived from data obtained at Children's Hospital Boston was first validated from data obtained at the University of California, San Francisco. Next, pooled data from the 2 institutions were analyzed, and a refined model was created using logistic regression methods. Finally, recursive partitioning was used to develop a clinically practical prediction tree to predict transcatheter systolic pressure gradient ≥ 20 mm Hg. Severe CoA was present in 48 patients (61%). Indexed minimal aortic cross-sectional area and heart rate-corrected flow deceleration time in the descending aorta were independent predictors of CoA gradient ≥ 20 mm Hg (p <0.01 for both). A prediction tree combining these variables reached a sensitivity and specificity of 90% and 76%, respectively. In conclusion, the presented prediction tree on the basis of cutoff values is easy to use and may help guide the management of patients investigated for CoA.

Diagnostic value of the flow profile in the distal descending aorta by phase-contrast magnetic resonance for predicting severe coarctation of the aorta.

PURPOSE: To compare aortic flow profiles at the level of the proximal descending (PDAo) and distal descending aorta (DDAo) in patients investigated for coarctation of the aorta (CoA), and compare their respective diagnostic value for predicting severe CoA. Diastolic flow decay in the PDAo predicts severe CoA, but flow measurements at this level are limited by flow turbulence, aliasing, and stent-related artifacts. MATERIALS AND METHODS: We studied 49 patients evaluated for CoA with phase contrast magnetic resonance imaging (PC-MRI). Parameters of diastolic flow decay in the PDAo and DDAo were compared. Their respective diagnostic value was compared with the standard reference of transcatheter peak gradient ≥20 mmHg. RESULTS: Flow measurement in the PDAo required repeated acquisition with adjustment of encoding velocity or location of the imaging plane in 69% of patients; measurement in the DDAo was achieved in single acquisition in all cases. Parameters of diastolic flow decay in the PDAo and DDAo, including rate-corrected (RC) deceleration time and RC flow deceleration yielded a good correlation (r = 0.78; P < 0.01, and r = 0.92; P < 0.01), and a similar diagnostic value for predicting severe CoA. The highest diagnostic accuracy was achieved by RC deceleration time at DDAo (sensitivity 85%, specificity 85%). CONCLUSION: Characterization of aortic flow profiles at the DDAo offers a quick and reliable noninvasive means of assessing hemodynamically significant CoA.

Advances in imaging: the impact on the care of the adult with congenital heart disease.

Recent advances in pediatric cardiology have dramatically changed the landscape of the field of congenital heart disease. This changing field is placing new demands on imaging to plan medical management as well as identify the need for, and timing of, reintervention. There are a number of imaging modalities available to the clinician when it comes to these evaluations, including echocardiography, computed tomography, and cardiac magnetic resonance imaging; each having their strengths and unique contributions. This article will discuss the advances in the aforementioned imaging modalities over the past decade and highlight how these tools can provide guidelines on the management of adults with congenital heart disease.