Short-Term Costs and Hospitalization Rates in Patients With Adult Congenital Heart Disease After Pulmonic Valve Replacement.

In the adult congenital heart disease (ACHD) population, pulmonary valve replacement (PVR) is a common intervention, its benefit, however, has been incompletely investigated. This study investigates short- and intermediate-term outcomes after PVR in ACHD. Using State Inpatient Databases from the Healthcare Cost and Utilization Project, we investigated both hospitalization rate and financial burden accrued over the 12-month period after PVR compared with the 12 months before. Among 202 patients who underwent PVR, per patient-year hospitalization rates doubled in the year after PVR compared with the year before (0.16 vs 0.36, p = 0.006). With the exception of postprocedural complications, the most common reasons for hospitalization were unchanged after surgery: 22% of patients were admitted with equal or greater frequency after PVR. These patients experienced higher inpatient costs both at index admission and in the year after PVR (p = 0.004 and p <0.001, respectively). Univariate predictors of increased hospitalizations after PVR were age ≥50 years (p = 0.016), transposition of the great arteries, or conotruncal abnormalities (p <0.001), lipid disorders (p = 0.025), hypertension (p = 0.033), and number of chronic conditions ≥4 (p = 0.004). Multivariate analysis identified transposition of the great arteries or conotruncal abnormalities as an independent risk factor for increased hospitalization and cost post-PVR (p ≤0.001). In conclusion, short-term costs and hospitalization rates increase after PVR in a small group of patients with ACHD.

Methodology for implementing patient-specific spatial boundary condition during a cardiac cycle from phase-contrast MRI for hemodynamic assessment.

Pulmonary insufficiency (PI) can render the right ventricle dysfunctional due to volume overloading and hypertrophy. The treatment requires a pulmonary valve replacement surgery. However, determining the right time for the valve replacement surgery has been difficult with currently employed clinical techniques such as, echocardiography and cardiac MRI. Therefore, there is a clinical need to improve the diagnosis of PI by using patient-specific (PS) hemodynamic endpoints. While there are many reported studies on the use of PS geometry with time varying boundary conditions (BC) for hemodynamic computation, few use spatially varying PS velocity measurement at each time point of the cardiac cycle. In other words, the gap is that, there are limited number of studies which implement both spatially- and time-varying physiologic BC directly with patient specific geometry. The uniqueness of this research is in the incorporation of spatially varying PS velocity data obtained from phase-contrast MRI (PC-MRI) at each time point of the cardiac cycle with PS geometry obtained from angiographic MRI. This methodology was applied to model the complex developing flow in human pulmonary artery (PA) distal to pulmonary valve, in a normal and a subject with PI. To validate the methodology, the flow rates from the proposed method were compared with those obtained using QFlow software, which is a standard of care clinical technique. For the normal subject, the computed time average flow rates from this study differed from those obtained using the standard of care technique (QFlow) by 0.8 ml/s (0.9%) at the main PA, by 2 ml/s (3.4%) at the left PA and by 1.4 ml/s (3.8%) at the right PA. For the subject with PI, the difference was 7 ml/s (12.4%) at the main PA, 5.5 ml/s (22.6%) at the left PA and 4.9 ml/s (18.0%) at the right PA. The higher percentage differences for the subject with PI, was the result of overall lower values of the forward mean flow rate caused by excessive flow regurgitation. This methodology is expected to provide improved computational results when PS geometry from angiographic MRI is used in conjunction with PS PC-MRI data for solving the flow field.

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.

Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment.

Adult patients with congenital heart disease (ACHD) represent a growing population due to progress in management. Surgical procedures generally fall short of restoring entirely normal anatomical and functional relations. Further procedures can be needed and lifelong follow-up is required. The right ventricle (RV) plays an important role in congenital heart disease and cardiac magnetic resonance (CMR) imaging has become the imaging method of choice for its assessment. CMR can provide relatively accurate measurements of RV volume and function, and arterial flow, with additional anatomical information provided by three-dimensional contrast angiography and late gadolinium imaging of fibrosis. Here we focus our review on three categories of ACHD in which evaluation of the RV is important: repaired tetralogy of Fallot, the systemic RV and Ebstein anomaly. We demonstrate how CMR contributes to decision-making regarding the types and timings of interventions. A dedicated CMR service should be regarded as a necessary facility of a centre specializing in the care of ACHD patients.

Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease.

Carcinoid disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells. It is the most common tumour affecting the small bowel. The majority of patients who progress to carcinoid syndrome develop cardiac disease selectively involving the right side of the heart, whereas left heart disease is unusual. The most common cause of death is dilatation and dysfunction of the right ventricle. Right ventricular dysfunction is largely secondary to pathological endocardial fibrosis of the tricuspid and pulmonary valves, presenting with regurgitation and stenosis. Average survival falls to only 11 months with the onset of symptoms, but recent evidence suggests that survival can be improved by early surgery in selected individuals. This article reviews the particular role that cardiovascular magnetic resonance imaging has in the management of carcinoid heart disease.

Quantitative assessment of pulmonary insufficiency by Doppler echocardiography in patients with adult congenital heart disease.

We determined the utility of continuous wave (CW) Doppler for quantification of pulmonary insufficiency (PI) confirmed by pulmonary angiography in patients with postoperative adult congenital heart disease. A total of 41 patients with PI were divided into two groups on the basis of PI severity by pulmonary angiography: group A (n = 27) with severe PI and group B (n = 14) with mild or moderate PI. Nine patients in group A had pulmonic valve replacement and reverted to mild PI after surgery. Their pre- and postoperative data were compared. All underwent a two-dimensional/Doppler study with interrogation of the PI jet for jet width by color Doppler and peak flow velocity, deceleration time (DT), pressure half-time (PHT), diastolic period (DP), and PI flow time (FT) by CW Doppler. The no-flow time (NFT), NFT/FT ratio, and NFT/DP fraction were calculated. Group A had a larger right ventricle (4.1 +/- 0.9 vs. 3.5 +/- 0.6 cm, P = .033), higher PI peak velocity (2.1 +/- 0.5 vs. 1.7 +/- 0.5 m/s, P = .04), shorter DT (261 +/- 61 vs. 317 +/- 83 ms, P = .018) and PHT (76 +/- 29 vs. 132 +/- 53, P < .0001), longer NFT (146 +/- 66 vs. 40 +/- 42 ms, P < .0001), and higher ratios of NFT/FT (46% +/- 27% vs. 13% +/- 14%, P < .0001) and NFT/DP (29% +/- 13% vs. 10% +/- 9%, P < .0001). The PHT and DT lengthened, and the NFT shortened in patients who underwent pulmonic valve replacement (all P < .05). By binary logistic regression, NFT and PHT were the best predictors for severe PI. An NFT of 80 ms had 84% sensitivity and 93% specificity, and a PHT of 100 ms had 93% sensitivity and 93% specificity for identifying angiographically severe PI. CW Doppler accurately distinguishes severe from lesser degrees of PI in patients with postoperative adult congenital heart disease.

Diastolic function in repaired tetralogy of Fallot at rest and during stress: assessment with MR imaging.

PURPOSE: To prospectively assess, with magnetic resonance (MR) imaging, right ventricular (RV) diastolic function after repair of tetralogy of Fallot (TOF) at rest and during pharmacologic stress and to study relationship between main pulmonary artery end-diastolic forward flow (EDFF) (indicative of restrictive RV physiology) and clinical status. MATERIALS AND METHODS: Institutional medical ethics committee approval and patient or parent informed consent were obtained. Patients with TOF corrected through the transatrial-transpulmonary approach underwent MR imaging at rest and during dobutamine stress and maximal exercise testing. Two-dimensional (2D) cine volumetric data were acquired. Flow measurements were performed with a standard 2D flow-sensitized sequence. MR imaging flow curves for tricuspid and pulmonary valves were combined into RV time-volume change curves, from which indexes of RV filling were derived. Patient results were compared with published data in control subjects. Student t tests, Mann-Whitney U tests, analysis of covariance, and paired and one-sample t tests were used. RESULTS: Thirty-six patients (mean age at repair, 0.9 year +/- 0.5 [standard deviation]; median age at study inclusion, 17 years [range, 7-23 years]; 26 male and 10 female patients) were included. Abnormalities in RV filling included impaired relaxation (prolonged deceleration time, P = .002; smaller early filling fraction, P = .02) in the entire group compared with published data in healthy control subjects and signs of restriction to RV filling (smaller atrial filling fraction and higher early filling/atrial filling peak ratio, P < .05 for both) in patients with EDFF (n = 24) compared with patients without EDFF (n = 12). Stress response was abnormal in patients with EDFF, who developed impaired RV relaxation not appreciated at rest. Patients with EDFF had more severe pulmonary regurgitation (P < .05) and poorer exercise performance (P < .001). CONCLUSION: In patients with TOF corrected with currently widely accepted surgical strategies, pulmonary artery EDFF relates to worse clinical state at mid- to long-term follow-up. Dobutamine stress imaging may unmask abnormalities in RV diastolic filling not appreciated with rest imaging alone.

Energy expenditure in infants with pulmonary insufficiency: is there evidence for increased energy needs?

The observed growth failure in infants with pulmonary insufficiency is postulated to be a consequence of elevated rates of energy expenditure. Assessment of energy expenditure by the classical technique of indirect calorimetry has yielded conflicting results. The adoption of the newer, doubly labeled water technique has provided evidence to support increased rates of energy expenditure in infants with chronic lung disease, congenital heart disease and in minimally ill, extremely low birth weight infants. The doubly labeled water technique holds great promise for the detailed study of energy expenditure in a variety of clinical conditions, including very ill as well as free-living subjects.