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.

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.

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.

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.

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.

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.

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.

Adult Tetralogy of Fallot: quantitative assessment of pulmonary perfusion with time-resolved three dimensional magnetic resonance angiography.

PURPOSE: To determine the potential role of dynamic temporally resolved three dimensional (3D) contrast-enhanced magnetic resonance angiography (MRA) for quantitative evaluation of pulmonary perfusion in adult patients with surgically treated Tetralogy of Fallot (ToF). MATERIALS AND METHODS: Institutional review board approval and written informed consent were obtained for this Health Insurance Portability and Accountability Act-compliant study. Thirty consecutive patients with surgically repaired ToF and 30 age-matched controls underwent breath-hold 3D time-resolved MRA (TR-MRA) and single-phase high-resolution 3D MRA of the thorax at 1.5 T. Two readers evaluated both datasets for image quality and findings. On TR-MRA datasets, regions-of-interest were placed over main pulmonary artery and lung fields obtaining signal intensity time curves. Using analytic software, time-to-peak (TTP), mean transit time (MTT), maximal signal intensity (MSI), maximum upslope of the curve (MUS), pulmonary blood volume (PBV), and pulmonary blood flow (PBF) were calculated. Pulmonary radionuclide scintigraphy was available for a subgroup of patients with ToF (n = 12). RESULTS: For ToF patients with unilateral pulmonary artery (PA) stenosis, TTP, and MTT were significantly longer, and MSI, MUS, PBV, and PBF were significantly lower in the ipsilateral lung compared with control subjects (P < 0.001 for all). There was no significant difference in TTP, MTT, MSI, MUS, PBV, and PBF between ToF patients without postsurgical stenotic residua and control subjects (P > 0.05 for all), nor between the mentioned perfusion indices for the contralateral lung in ToF patients with unilateral PA stenosis and control subjects (P > 0.05 for all). In ToF, patient with unilateral PA stenosis, analysis of contralateral-to-ipsilateral lung perfusion ratios on radionuclide scintigraphy and TR-MRA revealed significant correlation (r = 0.96). Bland-Altman plot showed a mean difference of 2.2% between the measured ratios (limits of agreement; -7.6%-12.0%). CONCLUSION: Time-resolved 3D contrast-enhanced MRA has potential for noninvasive and quantitative assessment of altered patterns of pulmonary perfusion in adult ToF, and may be a reliable technique for evaluation of postsurgical residua in these patients.

Revista Vídeo

Aborda os seguintes temas: aneurisma de ramo direito de artéria pulmonar com trombo; aneurisma e perfuração do folheto anterior da valva mitral secundário a endocardite da valva aórtica; obstrução de ventrículo direito devido a bandas musculares anômalas (dupla câmara de VD); dissecação aórtica com insuficiência aórtica secundária à protusão do “flap” por meio das cúspides da valva aórtica; rabdomioma de ventrículo direito em paciente com esclerose tuberosa; estenose pulmonar infundíbulo-valvar com forâmen oval patente

Assessment of pulmonary venous stenosis after radiofrequency catheter ablation for atrial fibrillation by magnetic resonance angiography: A comparison of linear and cross-sectional area measurements.

One of the recognised complications of catheter ablation is pulmonary venous stenosis. The aim of this study was to compare two methods of evaluation of pulmonary venous diameter for follow-up assessment of the above complication: (1) a linear approach evaluating two main diameters of the vein, (2) semiautomatically measured cross-sectional area (CSA). The study population consists of 29 patients. All subjects underwent contrast-enhanced magnetic resonance angiography (CeMRA) of the pulmonary veins (PVs) before and after the ablation; 14 patients were also scanned 3 months later. PV diameter was evaluated from two-dimensional multiplanar reconstructions by measuring either the linear diameter or CSA. A comparison between pulmonary venous CSA and linear measurements revealed a systematic difference in absolute values. This difference was not significant when comparing the relative change CSA and quadratic approximation using linear extents (linear approach). However, a trend towards over-estimation of calibre reduction was documented for the linear approach. Using CSA assessment, significant PV stenosis was found in ten PVs (8%) shortly after ablation. Less significant PV stenosis, ranging from 20 to 50% was documented in other 18 PVs (15%). CeMRA with CSA assessment of the PVs is suitable method for evaluation of PV diameters.

Doppler-echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot: comparison with cardiovascular magnetic resonance imaging.

AIMS: The purpose of this study was to compare the noninvasive assessment of severity of pulmonary regurgitation with Doppler echocardiography versus cardiovascular magnetic resonance imaging (CMR) in adult patients with repaired tetralogy of Fallot (rTOF). METHODS: We studied 52 (22 females) consecutive patients (aged 32 +/- 2 years, 23 +/- 5 years after rTOF) using Doppler echocardiography and compared these findings with CMR. From the continuous-wave Doppler trace, the duration of pulmonary regurgitation and of total diastole was measured and the ratio between the 2 was defined as pulmonary regurgitation index (PRi). Pulmonary regurgitant fraction (PRF) was assessed with flow phase velocity mapping with CMR. RESULTS: Patients were divided into 2 groups according to the median value (24.5%) of PRF measured by CMR: Group I (26 patients) with PRF < or =24.5% and Group II with PRF >24.5%. There was no difference between patients' age, sex, or age at repair between the 2 groups. More patients from Group II had a right ventricular outflow or transannular patch repair compared to Group I (12/26 [46%] vs 6/26 [23%], P <.01). Mean pulmonary regurgitation time was shorter (340 +/- 60 vs 440 +/- 135 ms, P =.001) and PRi was lower (0.61 +/- 0.11 vs 0.91 +/- 0.11, P <.001) in Group II compared to Group I. Color Doppler regurgitant jet was also broader in Group II (1.4 +/- 0.4 vs 0.7 +/- 0.5 cm, P <.001), signifying more severe pulmonary regurgitation. Doppler-measured PRi correlated closely with CMR regurgitant fraction (r = -0.82, P <.001) and with color Doppler pulmonary regurgitant jet width (r = -0.66, P <.001); the latter correlated with PRF assessed with CMR (r = 0.72, P <.001). A PRi <0.77 had 100% sensitivity and 84.6% specificity for identifying patients with pulmonary regurgitant fraction >24.5%, with a predictive accuracy of 95%. Furthermore, echocardiographically-assessed right ventricular end-diastolic dimensions correlated with CMR end-diastolic volume index (r = 0.49, P <.001 ). CONCLUSIONS: Pulmonary regurgitation is common in asymptomatic adults with rTOF. Severity of pulmonary regurgitation and its effects on right ventricular dimensions in these patients can be assessed noninvasively by Doppler echocardiography and CMR, with reasonable agreement between the 2 techniques.

[Complex assessment of the electrophysiological changes in patients with right-ventricular cardiac loading]. / Kompleksna otsenka na elektrofiziologichnite promeni pri bolni s desnokamerno obremeniavane na surtseto.

In 216 patients with right ventricular cardiac overload the changes of 76 electrophysiological indices were studied--indices of the corrected orthogonal electrocardiogram after Frank, corrected orthogonal vectorcardiogram, spatial electrocardiogram and high-speed spatial electrocardiogram. The patients with right ventricular cardiac overload are divided into two basic groups--patients with systolic and patients with diastolic overload. The indices are assessed on the basic criteria of sensitivity, specificity and realization. Variation analysis as well as statistical comparison with 119 healthy persons are performed. On the basis of the results of the study "sets" of 4 indices each with 97-98% sensitivity are determined, indices from all methods used are included in the "sets". The conclusion is reached of the good possibilities of the electrophysiological methods when a combination of indices with the help of a computer is made.

Assessment of residual right ventricular outflow tract obstruction following surgery using the response to intravenous propranolol.

The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.

Use of Doppler techniques (continuous-wave, pulsed-wave, and color flow imaging) in the noninvasive hemodynamic assessment of congenital heart disease.

Doppler echocardiography is a relatively new technique that has become an integral part of the cardiovascular ultrasound examination. The hemodynamic information provided by the Doppler technique is complementary to the tomographic anatomy depicted by the two-dimensional examination and, in some patients, may obviate the need for cardiac catheterization. In this article, we focus on the role of Doppler echocardiography in the noninvasive diagnosis of congenital cardiac abnormalities.

Assistência de enfermagem na dilatação da estenose pulmonar valvar / Nursing care to patient during dilatation of valvate pulmonary stenosis

O presente trabalho visa fornecer esclarecimentos sobre anatomia e patologia da estenose pulmonar valvar e sobre o seu trabalho. É dada ênfase especial à dilatação realizada na hemodinâmica com o uso de catéter- balão e à assistência de enfermagem prestada antes, durante e após o procedimento. (AU)

This article approaches the anatomy, pathology and treatment of valvate pulmonary stenosis. There is description of the dilatation in the hemodynamic area using balloon-catheter, the nursing care given during pre trans and post-operative period. (AU)

Modified Blalock-Taussig shunt operation without cardiac catheterization: two-dimensional echocardiographic preoperative assessment in cyanotic infants.

Between May 1981 and December 1983, 25 infants with cyanotic congenital heart defects underwent 26 Blalock-Taussig shunt operations without cardiac catheterization and angiocardiography. In these infants, the diagnosis was established by 2-dimensional echocardiography (2-D echo) supplemented with clinical findings, chest x-ray and electrocardiography. The right and left pulmonary artery measurements, a prerequisite for a shunt operation, determined by 2-D echo were compared with those at surgery and had an excellent correlation (r = 0.94). No infant died as a consequence of an early shunt failure, and only 1 died of postoperative pyothorax. In conclusion, 2-D echo may eliminate the need for invasive investigation in selected patients undergoing the Blalock-Taussig anastomosis.

Noninvasive assessment of valve lesions with Doppler ultrasound.

Noninvasive assessment of valvular lesions with Doppler echocardiography is based on determination of velocities of blood flow in the region of cardiac valves, adjacent cardiac chambers and in the large vessels. Obstructions lead to an increase in the velocity of flow in the region of the stenosis which can be registered with the Doppler technique. Through application of the Bernoulli equation, from the maximal velocity, the pressure gradient across the stenotic valve can be calculated. Additionally, the severity of the stenosis is reflected in the temporal course of the velocity curve of the jet through the stenosed valve. For this purpose, in mitral stenosis, the pressure half-time is employed and, in aortic stenosis, the peak of the velocity curve during systole is used. The severity of tricuspid and pulmonic stenosis can also be classified with a method analogous to that used in obstruction of the left heart. The diagnosis of valvular incompetence is based on the detection of regurgitant flow. The extent of regurgitant flow into the proximal cardiac chamber enables semiquantitative classification of severity. The intensity of the jet through the incompetent valve is also indicative of the size of the regurgitant volume. Similar to that in obstructive lesions, the temporal course of the velocity curve is also related to the severity. In association with high-grade regurgitant lesions, there is a premature decrease in the velocity curve. Additionally, the severity of aortic regurgitation can be assessed on the basis of the extent of regurgitant flow in the descending aorta or the subclavian arteries.(ABSTRACT TRUNCATED AT 250 WORDS)