64-Slice ECG-gated computed tomographic angiography for assessment of coronary arteries in brachycephalic dogs with pulmonary stenosis.

BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. METHODS: An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. RESULTS: All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. LIMITATION: Coronary artery morphology was not confirmed via postmortem examination in all patients. CONCLUSION: ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.

Integrative echocardiographic assessment of post-operative obstruction severity and restenosis after balloon valvuloplasty in 81 dogs with pulmonary stenosis.

INTRODUCTION/OBJECTIVES: Pulmonary stenosis (PS) is a congenital defect in the dog that is managed with balloon valvuloplasty (BV). Obstruction severity is routinely assessed using transthoracic echocardiography. The objectives of this study were to describe the short-term changes and long-term restenosis by retrospectively evaluating flow-dependent and flow-independent echocardiographic variables for dogs with PS after BV. ANIMALS, MATERIALS, AND METHODS: Medical records and stored echocardiographic images were reviewed from dogs that received BV for PS. The following echocardiographic variables were measured or calculated: maximum systolic ejection velocity (VmaxPV) and velocity-derived maximal pressure gradient (PV maxPG) across the pulmonary valve; ratio of aortic to pulmonary velocity time integral (VTIAV/VTIPV); ratio of aortic to pulmonary maximal velocity. RESULTS: Eighty-one dogs with PS that had a BV were included. Each of these dogs had pulmonary obstruction severity assessed in at least three timepoints. Forty-nine dogs had at least one additional examination performed, the last of which occurred a median of 504 days after the BV. Echocardiographic variables at the first follow-up had increased by a clinically relevant quantity in 20-34% of the dogs when comparing to the immediate post-BV examination. The last follow-up examination identified valve restenosis in 18-38% of the dogs in this study. Valve type and pre-BV obstruction severity did not have a significant effect (P > 0.1). CONCLUSIONS: For the assessment of BV efficacy, the immediate post-BV echocardiogram may not be as useful as the first follow-up examination several months later. Pulmonary restenosis after BV may be more prevalent than previously reported.

Echocardiographic assessment of right heart size and function in dogs with pulmonary valve stenosis.

INTRODUCTION/OBJECTIVES: We sought to determine the prevalence and clinical significance of right heart remodeling and right ventricular (RV) dysfunction in dogs with pulmonary valve stenosis (PS). We also sought to evaluate repeatability of several measurements of severity of PS, right heart size, and RV function in dogs with PS. ANIMALS, MATERIALS AND METHODS: Several indices of right atrial (RA) size and RV size and function were prospectively evaluated in 48 dogs with PS. Regression analysis was used to determine if indices of right heart size and function were independently associated with maximum transpulmonary pressure gradient (max PG) and adverse clinical findings (exercise intolerance, syncope, or right heart failure). Eight dogs underwent a second echocardiogram performed by the same operator to assess repeatability of the echocardiographic indices, which was quantified by coefficient of variation (CV) and repeatability coefficient. RESULTS: Increased RA size (81%), increased RV wall thickness (83%), and decreased tricuspid annular plane systolic excursion (TAPSE [81%]) were common. Right atrial size, end-diastolic RV area, and RV wall thickness were independently associated with max PG. Decreased TAPSE was independently associated with adverse clinical findings. All indices except RA area (18.6%) and RV systolic velocity (20.7%) had CVs <15%. Repeatability coefficients are available to help distinguish a true change versus measurement variability during serially obtained exams. CONCLUSIONS: Right heart remodeling and RV dysfunction are common in dogs with PS and are associated with echocardiographic and clinical severity. Results support the quantitative assessment of right heart size and function in dogs with PS.

Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair.

BACKGROUND: Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS). METHODS: A retrospective review was performed of all patients with TOF and pulmonary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early childhood (<8 years). Student's t test was performed to compare patients who underwent valve sparing (VS) versus transannular patch (TAP) repair. ANOVA was used to track measures of ventricular systolic function over time. RESULTS: We analyzed 151 echocardiograms performed on 42 patients. Pulmonary regurgitation (PR, moderate or severe) and the RV to left ventricular (LV) basal dimension ratio were higher in TAP patients (P < .04 at all-time points). Along with a significant increase in RV basal diameter Z-score in the TAP group (P < .001), there was an improvement in RV and LV GLS over time in both groups (P < .001). The LV GLS at last follow-up was lower in patients who underwent reoperation than those who did not (P = .050). LV GLS at the last follow-up echocardiogram was lower in patients with significant PR than those without (P < .001). CONCLUSIONS: Ventricular function appeared improve over time from the initial postoperative period in TOF patients. TAP repair was associated with a progressively higher RV/LV ratio in young children. GLS and RV/LV basal diameter ratio may be useful when following young children after TOF repair. Further research is necessary to understand the trajectory of ventricular functional and volumetric changes in young children in order to provide the most effective lifetime management of patients with TOF.

3D-assessment of RVOT dimensions prior percutaneous pulmonary valve implantation: comparison of contrast-enhanced magnetic resonance angiography versus 3D steady-state free precession sequence.

To compare contrast-enhanced magnetic resonance angiography (ceMRA) and 3D steady-state free precession (SSFP) during systole and diastole for assessment of the right ventricle outflow tract (RVOT) in patients considered for percutaneous pulmonary valve implantation (PPVI) after tetralogy of Fallot (TOF) repair. We retrospectively evaluated 89 patients (male: 45, mean age 19 ± 8 years), who underwent cardiac-MRI after surgical TOF-repair. Datasets covering the whole heart in systole and diastole were acquired using ECG-gated 3D SSFP and non-gated ceMRA. Measurements were performed in SSFP-sequences and in ceMRA in the narrowest region of the RVOT to obtain the minimum, maximum and effective diameter. Invasive balloon sizing as the gold standard was available in 12 patients. The minimum diameter in diastolic SSFP, systolic SSFP and ceMRA were 21.4 mm (± 6.1 mm), 22.6 mm (± 6.2 mm) and 22.6 mm (± 6.0 mm), respectively. Maximum diameter was 29.9 mm (± 9.5 mm), 30.0 mm (± 7.0 mm) and 28.8 mm (± 8.1 mm) respectively. The effective diameter was 23.2 mm (± 5.7 mm), 27.4 mm (± 6.7 mm) and 24.4 mm (± 6.2 mm), differing significantly between diastole and systole (p < 0.0001). Measurements in ECG-gated SSFP showed a better inter- and intraobserver variability compared to measurements in non-ECG-gated ceMRA. Comparing invasive balloon sizing with our analysis, we found the highest correlation coefficients for the maximum and effective diameter measured in systolic SSFP (R = 0.99 respectively). ECG-gated 3D SSFP enables the identification and characterization of a potential landing zone for PPVI. The maximum and effective systolic diameter allow precise sizing for PPVI. Patients with TOF-repair could benefit from cardiac MRI before PPVI.

Diagnostic Value of Contrast-Enhanced Multiphase Computed Tomography for Assessment of Percutaneous Pulmonary Valve Obstruction.

Percutaneous pulmonary valve replacement has emerged as an alternative to operation for some patients with congenital heart disease requiring intervention. Endocarditis is increasingly described as an adverse event during follow-up [1-4]. Diagnosis is difficult because of the poor visualization of the prosthetic valve in the pulmonary position by transthoracic and transesophageal echocardiogram and by the metallic artifact that degrades the image quality of magnetic resonance imaging (MRI). Two cases of percutaneous pulmonary valve obstruction diagnosed by cardiac computed tomographic angiography are presented. These cases demonstrate the utility of cardiac computed tomography for noninvasive imaging of suspected thrombus or endocarditis in a percutaneously placed pulmonary valve.

Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome.

OBJECTIVE: To review prevalence, management and prognostic factors of pulmonary stenosis (PS) in monochorionic diamniotic (MCDA) pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: Retrospective study over the last 10 years in a single referral center. We reviewed fetal echocardiography data of all MC twin cases with diagnosis of isolated PS. We assessed fetoscopy characteristics of those that underwent laser coagulation. We collected data regarding perinatal outcome, neonatal echocardiography and cardiac management. RESULTS: We found 24 cases of isolated PS among 2091 MCDA pregnancies. Among 1052 complicated MCDA that underwent fetal laser surgery, 22 (2.09%) developed PS of which 20 were diagnosed prenatally. Two cases were diagnosed in uncomplicated MCDA pregnancies (0.2%). Four of 22 (18.18%) cases with TTTS showed in utero regression after laser treatment. Thirteen newborns (65%) required valvular dilatation. Peak systolic velocities in the pulmonary artery trunk (PSV-PA) at diagnosis and the interval between the diagnosis of TTTS and that of PS were significantly different (p < 0.001 and p = 0.05 respectively) between PS requiring cardiac intervention and those who did not. CONCLUSION: An elevated PSV-PA at the time of PS diagnosis and a short time-interval between fetoscopic laser surgery and PS diagnosis are predictive of the need for interventional treatment after birth.

Three-dimensional rotational angiography in the assessment of vascular and airway compression in children after a cavopulmonary anastomosis.

The aim of the study was to examine the role of three-dimensional rotational angiography (3DRA) in assessing vascular and airway narrowing in children with a bidirectional cavopulmonary anastomosis (BCPA). The course of children with single ventricle physiology is often complicated by left pulmonary artery (LPA) and/or bronchial stenosis and may be related to aortic compression. 3DRA may be useful in evaluating this complex anatomy and possible mechanisms for the observed obstruction. Clinical data and imaging (2D angiography and 3DRA) of children with a BCPA were reviewed retrospectively. Measurements were taken at similar locations along the pulmonary arteries in both modalities and in the airways on 3DRA. Twenty-five children with a previous BCPA were assessed at mean age of 3.1 ± 2.0 years and weight of 13.6 ± 3.6 kg. Excellent correlation was found between 3DRA and 2D angiographic LPA measurements (r = 0.89, p < 0.0001). Twelve children had qualitative LPA stenosis on 3DRA, with a stenotic dimension of 6.6 ± 2.2 mm on 2D angiography and 6.8 ± 1.9 mm on 3DRA (r = 0.94, p < 0.0001). Ten cases with LPA stenosis also had bronchial stenosis (83 %). Qualitative airway assessment correlated with quantitative bronchial dimensions from 3DRA-derived tomographic images: Bronchial stenosis measured 4.4 ± 1.6 versus 5.9 ± 1.1 mm in those with a normal appearing bronchus (p = 0.009). Hybrid patients (initial palliation with bilateral pulmonary artery banding and arterial ductal stenting, n = 5) and all patients with a Damus-Kaye-Stansel (DKS) anastomosis (n = 9) were more likely to have LPA and left bronchial stenosis (OR 7.7, p = 0.04). 3DRA is a useful and accurate tool in assessment of LPA and airway narrowing after BCPA. Hybrid and DKS patients are more prone to LPA and bronchial stenosis, and 3DRA can provide insight into the mechanism.

Stent de platino Cheatham® para estenosis de arteria pulmonar en pacientes pediátricos / Cheatham® platinum stent forpulmonary artery stenosis in pediatric patients

CONTEXTO CLÍNICO: La estenosis de la arteria pulmonar o de sus ramas (EAP) representa el 2-3% de todas las lesiones cardiacas congénitas, siendo la cuarta causa más común de cardiopatía congénita (CC) en el mundo. La EAP se presenta generalmente asociada a otros defectos como la tetralogía de Fallot, atresia pulmonar, tronco arterioso, estenosis de válvula pulmonar y conducto arterioso persistente entre otros. Esta condición provoca una sub-perfusión del pulmón afectado y el consecuente desarrollo de hipertensión pulmonar en el vaso contralateral, causando disfunción del ventrículo derecho, arritmia y eventual muerte del paciente de no ser tratada. Las opciones usuales de tratamiento para EAP son la angioplastia con balón con o sin la colocación de un stent en la arteria pulmonar afectada. La angioplastia es utilizada en pediatría desde hace más de 20 años siendo el tratamiento de elección en este tipo de CC, reservándose la cirugía sólo en aquellos pacientes en los que no es posible realizarla. Habitualmente para este procedimiento se utiliza el stent de acero inoxidable Palmaz® (Johnson & Johnson), sin embargo este ha sido cuestionado por probables complicaciones derivadas de la rigidez de su composición. Actualmente existen otras alternativas de stents entre las que se encuentra el stent de platino Cheatham® (PC). La decisión del tipo de stent a utilizar depende de la edad y el tamaño del paciente, las dimensiones de la estructura vascular que se espera en la edad adulta, la morfología de la lesión, la necesidad probable de futuras intervenciones así como intervenciones previas. Se postula el uso del stent PC en pacientes pediátricos con diagnóstico de EAP, dado que presentaría potenciales beneficios derivados de su composición de platino menos rígida y que se podría adaptar mejor al crecimiento del paciente, comparado con otros tipos de stent usados convencionalmente. TECNOLOGÍA: El stent de platino Cheatham ® (PC) fue diseñado en 1996 para tratar pacientes con coartación de aorta, siendo posteriormente utilizado para otras indicaciones como la EAP. Está compuesto por un alambre de aleación de platino e iridium que se puede doblar y unir a la malla, logrando un stent de puntas redondeadas que puede dilatarse entre 8 y 25 mm. La posibilidad de dilatación a un diámetro grande, ha permitido la extensión de su uso a otras patologías pediátricas como la estenosis de la arteria pulmonar. Puede utilizarse como stent no recubierto o como stent cubierto con politetrafluoroetileno. OBJETIVO: Evaluar la evidencia disponible acerca de la eficacia, seguridad y aspectos relacionados a las políticas de cobertura de la utilización del stent de platino Cheatham ® para estenosis de la arteria pulmonar y sus ramas en pacientes pediátricos. MÉTODOS: Se realizó una búsqueda en las principales bases de datos bibliográficas (incluyendo Medline, Cochrane y CRD), en buscadores genéricos de Internet, agencias de evaluación de tecnologías sanitarias y financiadores de salud utilizando la siguiente estrategia de búsqueda: (CP Stent [all] OR Cheatham Platinum [all]) OR ((Pulmonary Artery [Mesh] OR Pulmonary Stenos*[tiab] OR Pulmonic Stenos*[tiab]) AND (CP Stent [tiab] OR Cheatham Platinum [tiab] OR Stents [Mesh] OR Stent*[tiab])). Se buscaron revisiones sistemáticas (RS), ensayos clínicos controlados aleatorizados (ECAs), estudios observacionales con más de un caso y con menos de 10 años de antigüedad, evaluaciones de tecnologías sanitarias (ETS) y económicas (EE), guías de práctica clínica (GPC) y políticas de cobertura de otros sistemas de salud cuando estaban disponibles. RESULTADOS: No se encontraron ECAs, ETS, EE, ni políticas de cobertura que mencionen el uso de esta tecnología. Se seleccionaron cuatro estudios observacionales y una GPC. CONCLUSIONES La evidencia encontrada es escasa y de muy baja calidad metodológica. No hay evidencia de que en pacientes con diagnóstico de estenosis de arteria pulmonar con o sin asociación a cardiopatías complejas, el stent Cheatham ® sea superior a otros stents utilizados habitualmente.

Functional and haemodynamic assessment of mild-to-moderate pulmonary valve stenosis at rest and during exercise.

OBJECTIVE: In adult patients with mild-to-moderate pulmonary valve (PV) stenosis, exercise capacity and haemodynamics have not been extensively studied, although regular exercise is recommended. Therefore, we aimed to assess exercise capacity to study the increase in PV gradient during exercise and to evaluate the impact of this increased pressure load on the RV. METHODS: Nineteen patients (8 female; 29±6.4 years) with isolated mild-to-moderate PV stenosis and no prior cardiac interventions were consecutively enrolled from the outpatient clinic of adult congenital heart disease. All patients underwent cardiopulmonary exercise testing, transthoracic echocardiography and bicycle stress echocardiography. Results for exercise testing were compared with age-matched and gender-matched control patients. RESULTS: In the studied population, resting heart rate (89±11 vs 75±14 bpm; p=0.001), peak power (199±66 vs 263±68 W; p=0.006); peak VO2 (31.2±9.9 vs 39±7.4 mL/kg/min; p=0.011); oxygen uptake efficiency slope (2430±913 vs 3292±943(mL/min)/(L/min); p=0.007) and VE/VCO2 slope (26.8±5.2 vs 22.6±4.3; p=0.01) differed significantly from controls. A linear increase of peak PV gradient with increasing flow was observed in the pooled dataset (Pearson's R=0.947; p<0.0001) and slopes identical as for control patients were obtained for the oxygen pulse-workload relationship. Right heart morphology and function were preserved in the studied patients. CONCLUSIONS: Patients with mild-to-moderate PV stenosis have decreased exercise capacity. A linear increase in PV gradient with flow suggests a fixed valve area throughout the exercise. Although systolic RV pressure load increases during exercise, good ventricular performance was observed without signs of functional or morphological changes of the right heart. CLINICAL TRIAL NUMBER: NCT01444222.

Utility of live/real time three-dimensional transesophageal echocardiography in the assessment and percutaneous intervention of bioprosthetic pulmonary valve stenosis.

Accurate echocardiographic evaluation of the pulmonary valve is technically difficult because of its close proximity to the left lung, which often limits decision making. Pulmonary valvotomy is the intervention of choice for symptomatic pulmonary valve stenosis, but fluoroscopy lacks appropriate real time anatomic detail. In this report, we present a case where direct imaging of the pulmonary valve with live/real time three-dimensional transesophageal echocardiography (3DTEE) aided in accurate evaluation and was then used to help guide and monitor successful valvuloplasty of a stenotic pulmonary valve bioprosthesis. We demonstrate that even in cases where two-dimensional (2D) evaluation of the pulmonary valve is difficult, the use of live/real time 3DTEE allows for accurate evaluation of bioprosthetic pulmonary valve structure and function, and enhances the precision and monitoring of percutaneous valvuloplasty.

Pulmonary artery pulsatility and effect on vessel diameter assessment in magnetic resonance imaging.

BACKGROUND: Information about thoracic vascular sizes can crucially affect clinical decision-making in cardiovascular disease. A variety of imaging techniques such as catheter angiography, contrast enhanced computed tomography (CT) and cardiac magnetic resonance imaging (CMR) are routinely used to measure vascular diameters. Traditionally, CMR black blood sequences were the main anatomical tool for visualization of vascular anatomy and still are in many centers. More recently, the vessel diameters are measured on multiplanar reconstructions derived from static magnetic resonance angiography (MRA). This study was performed to investigate the variation of vessel diameter measurements on different CMR techniques with respect to their data acquisition scheme. METHODS: We recruited two groups of patients for this prospective study. One group included patients with repaired tetralogy of Fallot (TOF), with at least moderate pulmonary insufficiency and another group of patients who underwent CMR as part of a diagnostic work-up for arrhythmogenic right ventricular cardiomyopathy (ARVC). Additional images of the right pulmonary artery (RPA) were acquired in the double inversion recovery (DIR) black blood, cine steady state free precession (SSFP) and MRA. All images were reviewed by two CMR trained readers using the electronic caliper provided within the picture archiving and communication system package. The maximum diameter of each artery was recorded in millimeters with up to one decimal point. Paired t-tests and Bland-Altman plots were used for comparison of measurements between different sequences. RESULTS: A total of 52 patients were recruited for this study, 26 patients in the TOF group (15 males, age 12.55±2.9) and 26 patients in the ARVC group (15 males, age 15.6±2.3). In both groups, the RPA sizes were not significantly different between the DIR images and diastolic cine SSFP (p>0.05). Measurements on DIR were significantly smaller than those made on systolic cine SSFP or MRA in both groups (p<0.0001). Vessel diameters measured on systolic SSFP were significantly larger than measurements made on diastolic SSFP (p<0.0001). There was no significant difference between vessel measurements on MRA and measurements on systolic cine SSFP (p=0.44 for the TOF group and p=0.79 for ARVC group). Measurements on the MRA datasets were significantly larger than those on diastolic SSFP images (p<0.0001). CONCLUSION: Black blood, white blood and MRA sequences are all reproducible CMR methods for the assessment of arterial diameters in children and adolescents. Measurements from systolic phase SSFP images are comparable to those from contrast-enhanced MRA. Therefore, the administration of contrast medium is not necessary in every case. Cine SSFP images offer the additional advantage over the other methods in such that both the largest and the smallest diameter of the vessel during the cardiac cycle can be assessed using this technique. This is even more relevant in highly pulsatile circulations, such as patients with repaired TOF and significant pulmonary insufficiency.

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.

Right ventricular outflow tract obstruction as a confounding factor in the assessment of the impact of pulmonary regurgitation on the right ventricular size and function in patients after repair of tetralogy of Fallot.

PURPOSE: To compare right ventricular (RV) size and function between patients with combined pulmonary regurgitation (PR) plus RV outflow tract (RVOT) obstruction (RVOTO) and patients with isolated PR. MATERIALS AND METHODS: Consecutive individuals with significant PR (PR fraction ≥ 20%) after tetralogy of Fallot (TOF) repair who underwent cardiovascular magnetic resonance (CMR) were included. Patients with additional hemodynamic abnormalities (residual ventricular septal defect, extracardiac shunt, and/or more than mild regurgitation at a valve other than the pulmonary valve) were excluded. Significant RVOTO was defined as peak gradient across RVOT ≥ 30 mmHg. RESULTS: Significant differences between patients with combined PR+RVOTO (n = 9) and isolated PR (n = 33) were observed in RV end-diastolic volume (138.6 ± 25.1 vs. 167.0 ± 34.6 mL/m(2) , P = 0.02, respectively), RV end-systolic volume (65.0 ± 9.6 vs. 92.7 ± 26.2 mL/m(2) , P = 0.003), and RV ejection fraction (RVEF) (52.8 ± 3.7 vs. 45.0 ± 6.4%, P = 0.001). Both PR and peak RVOT gradient were independent predictors of RV size. CONCLUSION: Patients with combined PR+RVOTO had smaller RV volumes and higher RVEF when compared with patients with isolated PR. The confounding effect of RVOTO on RV size and function needs to be considered in CMR studies evaluating patients after TOF repair.

Quantitative assessment of pulmonary regurgitation in patients with and without right ventricular tract obstruction.

BACKGROUND: There are concerns whether there is a difference in clinical utility of pulmonary regurgitation (PR) fraction (PRF) and PR volume (PRV) in subgroups of patients with isolated PR and individuals with combined PR and right ventricular outflow tract obstruction (RVOTO). The aim of the study was to compare PRF and PRV in patients with or without RVOTO. METHODS AND RESULTS: 82 consecutive patients after repair of tetralogy of Fallot (TOF) who underwent cardiovascular magnetic resonance and echocardiography were studied. There was no difference in PRF between patients with moderate and severe right ventricular (RV) dilatation (32±13% vs. 37±12%; p=0.18). Significant difference in PRV was observed between these groups (23±10 ml/m2 vs. 31±12 ml/m2, respectively; p=0.02). PRV had better ability than PRF in identification of severe RV dilatation, both in group with RVOTO [area under the curve (AUC) 0.82 vs. 0.72, p=0.005] and in patients without RVOTO (AUC 0.83 vs. 0.77, p=0.04). A strong correlation was seen between PRF and PRV both in patients with and without RVOTO [r=0.93, p<0.0001 and r=0.92, p<0.0001, respectively]. In both subgroups high variability of PRF was found in subjects with similar degree of PRV. CONCLUSIONS: PRV shows better ability than PRF in evaluating influence of PR on RV in patients after TOF repair, both in population with and without concomitant RVOTO.

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.

Valvular and hemodynamic assessment with CMR.

Cardiovascular magnetic resonance is able to provide a comprehensive assessment of valvular and hemodynamic function, including quantification of valve regurgitation and other flows, and accurate cardiac volumes and mass for assessing the effect on both ventricles. Combined with the ability to image all areas of the heart (including difficult areas, such as the right ventricle and pulmonary veins), it is an ideal technique for investigating patients who have heart failure in whom these areas need to be examined.

Usefulness of NT-proBNP in assessment of right ventricular function in children after tetralogy of Fallot correction - a preliminary study.

BACKGROUND: Although surgical treatment for tetralogy of Fallot (TOF) has been used with considerable success, right ventricular function may remain altered after repair. The NT-proBNP assessment has been shown to be a reliable parameter for the heart failure assessment. AIM: To determine NT-proBNP values in assessment of right ventricular function in children after TOF correction. METHODS: In 20 patients after TOF correction aged from 10 to 17 years (follow-up period ranged from 7 to 16 years) NT-proBNP level at rest and after exertion, treadmill test and echocardiography were performed. In the control healthy children NT-proBNP level at rest was assessed. RESULTS: The mean values of NT-proBNP level in the TOF patients were significantly higher than in controls (11.0 +/- 12.0 fmol/l and 5.4 +/- 7.5 fmol/l, p < 0.05). In patients repaired with a transannular patch the mean value of NT-proBNP level was higher than in children operated on without a transannular patch (18.3 +/- 16.5 vs. 6.8 +/- 7.9 fmol/l, p < 0.05). In children in whom physiological shortening of QRS complex during treadmill test was observed, NT-proBNP level was lower (mean values at rest 5.0 +/- 4.8 fmol/l and after exertion 7.3 +/- 6.3 fmol/l) compared to patients with prolongation of QRS duration (mean values at rest 17.7 +/- 15.6 fmol/l and after exertion 20.3 +/- 17.8 fmol/l) (p < 0.05). Significant differences in NT-proBNP levels between children with severe pulmonary regurgitation and mild/moderate pulmonary regurgitation were detected (mean values at rest 18.6 +/- 15.0 vs. 4.2 +/- 3.9 fmol/l and after exertion 20.0 +/- 18.6 vs. 5.7 +/- 4.6 fmol/l) (p < 0.05). The NT-proBNP levels were also higher in children with severe tricuspid valve insufficiency compared to children with mild/moderate tricuspid valve regurgitation (mean values at rest 19.5 +/- 15.0 vs. 4.9 +/- 3.7 fmol/l and after exertion 22.5 +/- 17.1 vs. 7.0 +/- 4.6 fmol/l). CONCLUSIONS: The NT-proBNP level in patients after TOF correction is higher than in healthy children. The NT-proBNP level is higher and exertion tolerance is lower in children repaired with rather than without transannular patch. In patients with severe pulmonary regurgitation and/or severe tricuspid valve insufficiency NT-proBNP level is higher than in patients without right ventricular volume overload. The measurement of NT-proBNP level might be helpful in order to separate those patients after TOF correction who are at increased risk of heart failure and arrhythmia.