Congenital polyvalvular disease expands the cardiac phenotype of the RASopathies.

The RASopathies are a group of similar genetic syndromes with cardiovascular abnormalities, characteristic facial features, short stature, abnormalities of the skin and musculoskeletal system, and variable neurodevelopmental challenges. The most common cardiovascular abnormalities include pulmonary valvular stenosis and hypertrophic cardiomyopathy. Congenital polyvalvular disease (CPVD) refers to congenital dysplasia of two or more cardiac valves. We diagnosed a RASopathy in two individuals with CPVD and noted that CPVD in RASopathies has rarely been reported in the literature. Thus, we performed a retrospective chart review and literature review to investigate the association and characterize the phenotype of CPVD in the RASopathies. CPVD was present in 2.5% (n = 6/243) of individuals in our RASopathy cohort. Involvement of two cardiac valves, commonly the aortic and pulmonic valves, was seen in the majority of individuals (6/8; 75%) in our cohort, but only 27% (3/11) of reported CPVD and RASopathy cases in the literature. CPVD should be considered an associated cardiovascular phenotype of the RASopathies, which has implications for diagnosis and management.

Differential contribution of the two waves of cardiac progenitors and their derivatives to aorta and pulmonary artery.

During mouse development, part of the cells derived from the second heart field (SHF) progenitors contributes to the elongation and enlargement of the outflow tract (OFT) that subsequently septates into the trunks of aorta (Ao) and pulmonary artery (PA). Thus, the cardiac progenitor-originated cells are distributed to both Ao and PA. Here, we investigated that how these cells are assigned to the two great arteries during OFT septation through lineage tracing technology. By use of the inducible Mef2c-AHF-CreERT2; Rosa26-mTmG reporter system, two waves of SHF progenitors and their derivatives were identified, and they made differential contribution to the Ao and PA, respectively. While the early wave of cells (at E7.5) was preferentially destined to the Ao, the second wave of cells (from E8.5 till E11.5) made its favorite path to the PA. In addition, we unveiled PDK1 as a critical regulator of the second wave of cells as deletion of Pdk1 resulted in poorly developed PA leading to pulmonary stenosis. Thus, this study provides insights into the understanding of the pre-determined cell fate of the cardiac progenitor-derived cells with preferential contribution to the Ao and PA, as well as of the pathogenesis of pulmonary stenosis.

Congenital heart disease in school children in Lagos, Nigeria: Prevalence and the diagnostic gap.

Congenital heart disease (CHD) in low-and-middle income countries (LMIC) is often characterized by late presentation resulting from inadequate screening and healthcare access in these regions. Accurate estimates of the burden of CHD among school children are often lacking. The objective of this study was to determine the prevalence and distribution of CHD among school children in two communities (urban and semi-urban) in south western Nigeria. Using clinical assessment and portable echocardiography, 4107 school children aged 5 years to 16 years in Lagos, Nigeria, were selected using a multistage sampling procedure and screened for CHD. Diagnosis of CHD was made after echocardiography. Children identified with CHD were referred to a tertiary hospital for appropriate cardiac care. The 4,107 children screened had a mean age of 11.3 ± 2.7 years and 53.7% were females. Twenty seven children had echocardiography-confirmed CHD, representing a prevalence of CHD among school children in Lagos, Nigeria of 6.6 per 1000 children. Acyanotic CHD constituted 96.3% of detected cases. Two children diagnosed with CHD (Tetralogy of Fallot and severe pulmonary valve stenosis respectively) had successful intervention. The prevalence of previously undiagnosed CHD among school children in Lagos Nigeria is substantial and highlights gaps in the health care system and school health programs. Echocardiographic screening of school children provides an opportunity for missed early diagnosis and treatment of CHD and reduces the prevalence of first-diagnosed CHD in adulthood. Therefore, focused clinical examination of school children followed by echocardiography is a strategy that could bridge this diagnostic and treatment gap in CHD.

Phenotypic delineation of the retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome.

Retinal arterial macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS), also known as Familial Retinal Arterial Macroaneurysms (FRAM) syndrome, is a very rare multisystem disorder. Here, we present a case series comprising ophthalmologic and systemic evaluation of patients homozygous for RAMSVPS syndrome causative IGFBP7 variant. New clinical details on 22 previously published and 8 previously unpublished patients are described. Age at first presentation ranged from 1 to 34 years. The classical feature of macroaneurysms and vascular beading involving the retinal arteries was universal. Follow up extending up to 14 years after initial diagnosis revealed recurrent episodes of bleeding and leakage from macroaneurysms in 55% and 59% of patients, respectively. The majority of patients who underwent echocardiography (18/23) showed evidence of heart involvement, most characteristically pulmonary (valvular or supravalvular) stenosis, often requiring surgical correction (12/18). Four patients died in the course of the study from complications of pulmonary stenosis, cerebral hemorrhage, and cardiac complications. Liver involvement (usually cirrhosis) was observed in eight patients. Cerebral vascular involvement was observed in one patient, and stroke was observed in two. We conclude that RAMSVPS is a recognizable syndrome characterized by a high burden of ocular and systemic morbidity, and risk of premature death. Recommendations are proposed for early detection and management of these complications.

One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy.

BACKGROUND: We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). Methods and Results: Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR. CONCLUSIONS: At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.

Infundibular Pulmonic Stenosis in a Moluccan Cockatoo (Cacatua moluccensis).

A 31-year-old female Moluccan cockatoo (Cacatua moluccensis) was examined for intermittent foot clenching of 4 months' duration. Physical examination revealed feather-destructive behavior and clinical findings compatible with hypovitaminosis A. Neurologic examination was unremarkable. Results of radiographs, hematologic testing, plasma biochemical analyses, and measurement of lead and trace element blood concentrations were unremarkable, except for degenerative joint disease of several thoracic intervertebral joints and a low blood copper concentration. Increased dietary copper was recommended. After a 6-month period without clinical signs, the bird presented again for episodes of foot weakness. Radiographic review was suggestive of mild pulmonary trunk enlargement. Echocardiography revealed mild mitral and aortic regurgitation, dilation of the ascending aorta, and a dilated right ventricle with turbulent right ventricular outflow. An electrocardiogram revealed a sinus rhythm and normal-appearing complexes. Nonselective fluoroscopic angiography was performed 3 weeks later because of persistent episodes of foot clenching and weakness. Infundibular pulmonic stenosis, poststenotic dilation of the pulmonic trunk, and proximal main pulmonary arteries were identified, as well as a mild narrowing of the descending aorta compatible with aortic stenosis. The bird was discharged without medication but with dietary recommendations and experienced 2 clenching episodes in the days after the last visit. No recurrence of clinical signs has been reported over the 18-month follow-up period. To our knowledge, this is the first report of infundibular pulmonic stenosis in a bird. This case illustrates the application of basic and advanced diagnostic imaging modalities in evaluating cardiac disease in birds.

Coronary artery ectasia in Noonan syndrome: Report of an individual with SOS1 mutation and literature review.

Noonan syndrome (NS) is the second most frequent hereditary syndrome with cardiac involvement. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most prevalent cardiovascular abnormalities. We report on a 14-year-old girl with NS due to SOS1 mutation with pulmonary stenosis and idiopathic coronary ectasia. To the best of our knowledge, this is the first report describing coronary ectasia in a patient with NS secondary to a SOS1 mutation. We include a literature review of this rare association.

The Effects of Hemodynamic Alterations on Lung Volumes in Fetuses with Tetralogy of Fallot: An MRI Study.

This study assessed whether the presence of tetralogy of Fallot (TOF) affects fetal lung development and whether these fetuses are at risk of pulmonary hypoplasia (PH). Furthermore, we investigated whether the degree of the concomitant pulmonary valve (PV) stenosis or a stenosis in the branch pulmonary arteries correlates with the fetal lung volume. Lung volumetry was performed in 16 fetuses with TOF who underwent MRI between gestational weeks 21 and 35 and in 22 controls. Fetal biometric data and the diameters of the PVs were evaluated by ultrasound. PV and branch pulmonary artery diameters were standardized (z-scores), and fetal lung volume/fetal body weight (FLV/FBW) ratios (ml/g) were calculated. The mean FLV/FBW ratio (0.031 ± 0.009 ml/g) in the TOF group was statistically significantly lower than in the control group (0.041 ± 0.009 ml/g; P = 0.003). None of the fetuses with TOF met the criterion for PH. FLV did not correlate with the degree of PV stenosis, but rather with the presence of a significant stenosis in at least one branch pulmonary artery. The presence of TOF moderately affects fetal lung growth, which is apparently not dependent on the degree of the PV stenosis. However, only an additional stenosis in at least one branch pulmonary artery was associated with a small FLV, but not with PH. Thus, reduced pulmonary blood flow may be offset by additional factors, such as the ability to establish a sufficient collateral system and to alter structural vascular size and, thus, pulmonary vascular resistance.

The impact of pulmonary insufficiency on the right ventricle: a comparison of isolated valvar pulmonary stenosis and tetralogy of fallot.

Pulmonary insufficiency (PI) is associated with right ventricular (RV) dilation, dysfunction, and exercise intolerance in patients with tetralogy of fallot (TOF). We sought to compare RV function and exercise performance in patients with valvar pulmonary stenosis (VPS) following pulmonary balloon valvuloplasty to those with repaired TOF with similar degrees of PI. We performed a cross-sectional study of patients with VPS and TOF. Cardiac magnetic resonance (CMR) and exercise stress test were performed. Subjects were matched by time from initial procedure and severity of PI using propensity scores. After matching, there were 16 patients with VPS and 16 with TOF for comparison, with similar demographics. Time from initial procedure was 14 years (12-16), p = 0.92, and pulmonary regurgitant fraction was 19 % (6-31), p = 0.94, Patients with TOF had lower ejection fraction [58 % (53-66) vs. 65 % (60-69), p = 0.04] and more RV hypertrophy [69 g/m(2) (52-86) vs. 44 g/m(2) (32-66), p = 0.04] compared to those with VPS. Aerobic capacity was worse in patients with TOF [68 ± 19 % mVO2 (56-84) vs. 82 ± 9.2 % (74-89) in VPS, p = 0.01], with a trend for less habitual physical activity [0.9 (0-12) vs. 8 h/week (4-12), p = 0.056], respectively. With similar degrees of PI, patients with TOF demonstrate worse RV function and aerobic capacity as compared to patients with just VPS. Habitual exercise may in part explain differences in exercise performance and should be further explored.

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.

CLINICAL VARIABILITY IN TWO SISTERS WITH KEUTEL SYNDROME DUE TO A HOMOZYGOUS MUTATION IN MGP GENE.

Keutel syndrome (KS) is an autosomal recessive disease characterised by abnormal cartilage calcification, brachytelephalangism, peripheral pulmonary artery stenosis, hearing loss and midface retrusion. KS is caused by homozygous mutations in MGP, a gene encoding Matrix Gla protein which acts as a calcification inhibitor in extracellular matrix. We present two Turkish sisters (22 and 13 years old) who had abnormal cartilage calcification, brachytelephalangism, congenital heart defect and chronic asthmatic bronchitis. The patients were homozygous for c.62-2A>G (IVS1-2 A>G) mutation in MGP gene. Abnormal cartilage calcification, brachytelephalangism and midfacial retrusion are the hallmarks of KS. It was observed that the younger sister had striking cartilaginous calcifications, midfacial retrusion and severe brachytelephalangism while her older sister had mild costal cartilaginous calcifications and brachytelephalangism without any midfacial retrusion. Intrafamiliar clinical variability for KS has not been described previously.

CT and MRI of pulmonary valvular abnormalities.

Pulmonary valve disease constitutes a wide spectrum of conditions. Traditionally, echocardiography has been the technique of choice for the evaluation of pulmonary and other valvular disease. However, with advances in technology, computed tomography (CT) and magnetic resonance imaging (MRI) are playing increasingly important roles in the evaluation of these disorders. In this article, we review the normal appearance of the pulmonary valve and then illustrate various variants and pathological entities of the pulmonary valve.

TGFß and Wnt in cardiac outflow tract defects in offspring of diabetic pregnancies.

BACKGROUND: Diabetes mellitus in pregnancy causes defects in infant heart, including the outflow tracts (OFTs). Development of the aorta and pulmonary artery, which are derived from the common OFT in the embryo, is regulated by the transforming growth factor ß (TGFß) and Wnt families, and can be perturbed by hyperglycemia-generated intracellular stress conditions. However, the underlying cellular and molecular mechanisms remain to be delineated. METHODS: Female mice were induced diabetic with streptozotocin. Embryonic and fetal OFTs were examined morphologically and histologically. Cell proliferation was assessed using 5'-bromo-2'-deoxyuridine incorporation assay. Oxidative and endoplasmic reticulum (ER) stress markers and TGFß factors were detected using immunohistochemistry. The expression of genes in the Wnt-signaling system was assessed using real-time reverse transcription polymerase chain reaction array. The role of activin-A in cell proliferation was addressed by treating embryos cultured in high glucose with activin-A. RESULTS: Maternal diabetes caused complex abnormalities in the OFTs, including aortic and pulmonary stenosis and persistent truncus arteriosus. The development of the endocardial cushions was suppressed, manifested with insufficient cellularization of the tissues. Cell proliferation was significantly decreased under oxidative and ER stress conditions. The expression of genes in the Wnt signaling was significantly altered. Activin-A and Smad3 were found to be expressed in the OFT. Treatment with activin-A rescued cell proliferation in the endocardial cushions. CONCLUSIONS: Maternal diabetes generates oxidative and ER stress conditions, suppresses TGFß and Wnt signaling, inhibits cell proliferation and cellularization of the endocardial cushions, leading to OFT septal defects. Activin-A plays a role in hyperglycemia-suppressed proliferation of the endocardial cells.

Analysis of an automated background correction method for cardiovascular MR phase contrast imaging in children and young adults.

BACKGROUND: Phase contrast magnetic resonance imaging (MRI) is a powerful tool for evaluating vessel blood flow. Inherent errors in acquisition, such as phase offset, eddy currents and gradient field effects, can cause significant inaccuracies in flow parameters. These errors can be rectified with the use of background correction software. OBJECTIVE: To evaluate the performance of an automated phase contrast MRI background phase correction method in children and young adults undergoing cardiac MR imaging. MATERIALS AND METHODS: We conducted a retrospective review of patients undergoing routine clinical cardiac MRI including phase contrast MRI for flow quantification in the aorta (Ao) and main pulmonary artery (MPA). When phase contrast MRI of the right and left pulmonary arteries was also performed, these data were included. We excluded patients with known shunts and metallic implants causing visible MRI artifact and those with more than mild to moderate aortic or pulmonary stenosis. Phase contrast MRI of the Ao, mid MPA, proximal right pulmonary artery (RPA) and left pulmonary artery (LPA) using 2-D gradient echo Fast Low Angle SHot (FLASH) imaging was acquired during normal respiration with retrospective cardiac gating. Standard phase image reconstruction and the automatic spatially dependent background-phase-corrected reconstruction were performed on each phase contrast MRI dataset. Non-background-corrected and background-phase-corrected net flow, forward flow, regurgitant volume, regurgitant fraction, and vessel cardiac output were recorded for each vessel. We compared standard non-background-corrected and background-phase-corrected mean flow values for the Ao and MPA. The ratio of pulmonary to systemic blood flow (Qp:Qs) was calculated for the standard non-background and background-phase-corrected data and these values were compared to each other and for proximity to 1. In a subset of patients who also underwent phase contrast MRI of the MPA, RPA, and LPA a comparison was made between standard non-background-corrected and background-phase-corrected mean combined flow in the branch pulmonary arteries and MPA flow. All comparisons were performed using the Wilcoxon sign rank test (α = 0.05). RESULTS: Eighty-five children and young adults (mean age 14 years; range 10 days to 32 years) met the criteria for inclusion. Background-phase-corrected mean flow values for the Ao and MPA were significantly lower than those for non-background-corrected standard Ao (P = 0.0004) and MPA flow values (P < 0.0001), respectively. However, no significant difference was seen between the standard non-background (P = 0.295) or background-phase-corrected (P = 0.0653) mean Ao and MPA flow values. Neither the mean standard non-background-corrected (P = 0.408) nor the background-phase-corrected (P = 0.0684) Qp:Qs was significantly different from 1. However in the 27 patients with standard non-background-corrected data, the difference between the Ao and MPA flow values was greater than 10%. There were 19 patients with background-phase-corrected data in which the difference between the Ao and MPA flow values was greater than 10%. In the subset of 43 patients who underwent MPA and branch pulmonary artery phase contrast MRI, the sum of the standard non-background-corrected mean RPA and LPA flow values was significantly different from the standard non-background-corrected mean MPA flow (P = 0.0337). The sum of the background-phase-corrected mean RPA and LPA flow values was not significantly different from the background-phase-corrected mean MPA flow value (P = 0.1328), suggesting improvement in pulmonary artery flow calculations using background-phase-correction. CONCLUSION: Our data suggest that background phase correction of phase contrast MRI data does not significantly change Qp:Qs quantification, and there are residual errors in expected Qp:Qs quantification despite background phase correction. However the use of background phase correction does improve quantification of MPA flow relative to combined RPA and LPA flow. Further work is needed to validate these findings in other patient populations, using other MRI units, and across vendors.

Williams-Beuren syndrome: computed tomography imaging review.

Williams-Beuren syndrome (WBS) affects young infants and children. The underlying etiopathogenesis of this rare disease is due to the mutation of the elastin gene that is responsible for the elasticity of the arterial wall. As a result of inadequate elastin production, the major systemic arteries become abnormally rigid and can be manifested by an impediment to the blood flow. The most common cardiovascular abnormalities encountered in WBS are supravalvular aortic stenosis, pulmonary arterial stenosis, and mitral valve prolapse. Less frequently observed cardiovascular abnormalities include coarctation of the aorta, ventricular septal defect, patent ductus, subaortic stenosis, and hypertrophic cardiomyopathy. Coronary artery stenosis and severe impediment to the bi-ventricular outflow as a result of supravalvular aortic and pulmonary artery stenosis predispose patients to sudden death. Patients with progressed arterial stenosis and severe stenosis are likely to require intervention to prevent serious complications. Rarely, imaging findings may precede clinical presentation, which allows the radiologist to participate in the patient care. However, to be more prudent, the radiologist must be accustomed to the imaging characteristics of WBS as well as the patient's clinical information, which could raise the suspicion of WBS. We performed a retrospective analysis of all the available images from patients diagnosed with WBS in last 4 years at our institution, and present key imaging findings along with a review of the literature to summarize the clinically relevant features as demonstrated by multidetector computed tomography in WBS. Cross-sectional imaging plays a vital role in the diagnosis of WBS cases with equivocal clinical features. MDCT evaluation of complex cardiovascular abnormalities of WBS including coronary artery disease is feasible with modern MDCT scanners and in the future, this approach could provide accurate information for planning, navigation, and noninvasive assessment of the secondary arterial changes in WBS and thus reducing the dependence upon invasive contrast catherization techniques.

Simultaneous transcatheter treatment of perimembranous ventricular septal defect and other congenital cardiopathies.

OBJECTIVE: To assess the efficacy and safety of simultaneous transcatheter corrections of perimembranous ventricular septal defect (VSD) and other congenital cardiopathies. PATIENTS/METHODS: From 2004 to 2012, 56 patients (25 male, 31 female), aged 14.2±10.1, with compound congenital cardiovascular abnormalities underwent simultaneous transcatheter interventional procedure. Of the 56 patients, 32 had VSD and atrial septal defects (ASD); 17 had VSD and patent ductus arteriosus (PDA); and seven had VSD and pulmonary valve stenosis (PS). Percutaneous balloon pulmonary valvuloplasty (PBPV) was performed before the closure of VSD, PDA, or ASD. RESULTS: The combined transcatheter interventional procedure was successfully performed in all patients. Among these, two occluders were implanted in each of 49 patients, seven patients with VSD combined with PS underwent successfully balloon valvuloplasty and VSD closure. The size of VSD, ASD and PDA detected by TTE was 4.8±1.7 mm, 9.0±5.0 mm and 4.5±2.5 mm, respectively. The occluder diameter of VSD, ASD and PDA was 7.6±2.2 mm, 14.3±6.2 mm and 7.9±3.2 mm, respectively. The peak-to-peak transpulmonary gradient decreased from 60.4±19.7 mmHg to 15.0±5.0 mmHg (p<0.001) in seven patients with VSD combined with PS. One patient with VSD and ASD had a permanent pacemaker implanted because of third-degree atrioventricular block two months after the procedure. There were not serious adverse events in relation to the combined procedures during the 23.8±20.7 months of follow-up in other 55 patients. CONCLUSION: The simultaneous treatment of VSD and other congenital cardiopathies using transcatheter-based procedures is safe and effective, which can provide satisfactory results.

Histoenzymological characteristics of the heart conduction system: comparative study with left or right ventricle afterload.

Histoenzymological changes, indicating inhibition of the main metabolic processes, were found in the conduction cardiomyocytes of the left ventricle and ventricular septum in experimental stenosis of the aorta. The histoenzymological changes in the conduction system of both ventricles and ventricular septum were similar in experimental stenosis of the pulmonary artery and indicated primarily activation of glycolysis. The histoenzymological profile of conduction cardiomyocytes differed little in cases when the increase of the pressure load was complicated or not complicated by the development of heart failure, particularly in pulmonary artery stenosis. The histoenzymological changes in the conduction system in response to increased afterload differed significantly from those in the contractile myocardium and correlated with the level of cellular functional activity and sensitivity to the regulatory and alterative exposure. These data attest to minor role of metabolic shifts in conduction cell injuries with increasing afterload, primarily, of the right ventricle.

[Hepatic structure remodeling after an experimental pulmonary trunk stenosis and following its operative correction].

Changes in the liver were studied in 25 puppies with experimental pulmonary trunk stenosis of 6-12 months duration and 10 animals after the elimination of this defect. Control group included 10 dogs of the corresponding age. A complex of histological, morphometric, electron microscopic and immunohistochemical methods was used. During the modeling of pulmonary trunk stenosis, the resistance of hepatic afferent vessels to hepatic blood flow was increased due to the venous-arterial and venous-venous reactions. In the arteries, the bundles of smooth myocytes (SM) of the intimal muscle were formed together with the musculo-elastic sphincters, polypoid cushions, while in the branches of the portal vein, the intimal SM bundles and the valves appeared. In the efferent veins, the muscular elevations were hypertrophied. In all the vessels the thickening of the walls was observed, and in the media of the arteries, there were signs of sclerosis and the increased expression of alpha-smooth muscle actin (alpha-SMA). Hepatocytes demonstrated marked ultrastructural changes: mitochondrial matrix swelling, partial destructions of their cristae, dilation of endoplasmic reticulum cisterns. After the elimination of the defect, previously formed vascular adaptation reactions were found to disappear, the tone of the blood vessels in the liver decreased, causing the regression of hypertrophic changes of their media. The number of the arterial blood vessels with intimal muscle, sphincters and cushions decreased. The expression of alpha-SMA in the media of the arteries was also reduced. In hepatic efferent veins, the muscular elevations became attenuated. The dystrophic changes in hepatocytes regressed at both light-microscopic and the ultrastructural level.

[Practical approach of cardiac valvulopathies]. / Approche des valvutlopathies cardiaques par le praticien. Investigations diagnostiques et place du traitement médicamenteux.

In the light of the recommendations published in 2012 by the European Society of Cardiology, the present article provides a review of the assessment, diagnosis and drug therapy of frequent cardiac valvular disease in adults. Congenital valvular heart disease, as well as pathology of the pulmonary valve and tricuspid stenosis, which are less frequent, will not be discussed here.

Valve-sparing options in tetralogy of Fallot surgery.

Given late outcomes of patients with tetralogy of Fallot repaired in the 1970s and 1980s, as well as a better understanding of the late deleterious effects of pulmonary regurgitation, there is a tendency toward preservation of the pulmonary valve function during primary repair of tetralogy of Fallot. The bar keeps moving downward, to include smaller and more dysmorphic pulmonary valves. This article reviews some useful indications and techniques for valve-sparing options, including intraoperative balloon dilation and cusp reconstruction using a patch. Just like other valve repair techniques, no one technique can be applied uniformly, and surgeons must master a wide armamentarium of techniques.