BACKGROUND: The survival of patients with neuroendocrine tumors has substantially improved with modern treatment options. Although the associated carcinoid syndrome can be diagnosed early and controlled effectively, cardiologists still encounter patients with cardiac manifestations, particularly among individuals with persistently high levels of vasoactive mediators. Treatment options have been limited to surgical valve replacement in fully manifested disease. Since surgery is not always feasible, transcatheter valve implantation is becoming an interesting alternative.
METHODS: A case of a 50-year-old woman with carcinoid syndrome and right-sided valvular heart disease is presented. Moderate pulmonary valve stenosis and severe tricuspid valve regurgitation were diagnosed during the evaluation and treatment of neuroendocrine tumor. The possibility of rare valve involvement and the need for interdisciplinary cooperation in the diagnosis, monitoring and treatment of patients with neuroendocrine tumors producing vasoactive substances must be emphasized.
CONCLUSIONS: The patient had a typically presenting carcinoid syndrome with a rare cardiac manifestation. Although monitoring and treatment were carried out in accordance with recommendations and appropriate to the clinical condition, rapid progression of the metastatic disease ultimately precluded invasive cardiac intervention.

There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of infundibular obstruction is seen in more severe PS cases and in older subjects. Reappearance of PS was observed in approximately 10 % of patients following BPV. The reasons for recurrence were found to be balloon/annulus ratio less than 1.2 and immediate post-BPV pulmonary valve peak gradients greater than 30 mmHg. Recurrent stenosis is successfully addressed by repeating BPV with lager balloons than used initially. Long-term results revealed continue relief of obstruction, but with development of pulmonary insufficiency, some patients requiring replacement of the pulmonary valve. It was concluded that BPV is the treatment of choice in the management valvar PS and that balloon/annuls ratio used for BPV should be lowered to 1.2 to 1.25. It was also suggested that strategies should be developed to prevent/reduce pulmonary insufficiency at long-term follow-up.

Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome.
Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute\'s Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal.
Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality.
The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon\'s technical expertise and well-structured hospital facilities.

Double-outlet right ventricle (DORV) is a rare cardiac condition in which both the aorta and pulmonary artery arise from the right ventricle, resulting in parallel systemic and pulmonary circulations. Usually, DORV is present with ventricular septal defect; however, the location of the ventricular septal defect and presence of pulmonary stenosis (PS) result in various physiological features and subtypes of DORV. Because DORV without PS causes congestive cardiac failure and DORV with PS results in cyanotic heart disease, anesthesia management varies widely according to the resultant physiological characteristics. Reports of anesthesia management in a parturient with DORV undergoing cesarean delivery is scarce because of the low incidence of DORV and the discouragement of these patients to conceive. Only 8 known previous such cases are reported, and almost all these patients were administered regional anesthesia. Here we describe a parturient with DORV, to whom general anesthesia was administered because of incidental antiphospholipid syndrome with low platelets. To the best of our knowledge, this scenario has not been described previously.

Williams syndrome (WS) is a rare congenital developmental disorder caused by the deletion of between 26 and 28 genes on chromosome 7q11.23. For patients with WS, in view of the particularity of the supravalvular aortic stenosis, choosing appropriate arterial cannula, maintaining higher perfusion pressure as well as strengthening myocardial protection during cardiopulmonary bypass (CPB) is essential to the clinical outcome. Here, we report a child with pulmonary artery valvular stenosis who failed to wean off CPB because of malignant arrhythmias and cardiac insufficiency after surgical correction of pulmonary valvular stenosis. With the assistance of extracorporeal membrane oxygenation (ECMO), emergency cardiac catheterization revealed supravalvular aortic stenosis (SVAS), which suggests a suspected missed diagnosis of WS. Finally, under the support of ECMO, the cardiac function gradually returned to normal, and the child was discharged 23 days after surgery.

Noonan syndrome (NS) is a genetic disorder characterized by facial dysmorphism, congenital heart disease, and short stature. In very rare cases, patients with this syndrome have coronary disease. Their management and prognosis are currently unclear. We have described 4 cases of coronary aneurysms/coronary ectasia and 1 case of a single coronary artery in patients with adult Noonan syndrome, followed in a medical and surgical center of adult congenital heart disease. The average age was 49.4years old. The majority of them had both pulmonary stenosis and interauricular communication. None had symptoms of angina, at rest, or with stress. Only one patient who had any structural heart disease, had a thrombotic complication with chronic occlusion of the right coronary and anterior inter ventricular artery, fortuitous finding, with no ischemic signs to functional tests, treated only with anti-vitamin K. Finally, any deaths have also been reported in our series. Coronary artery diseases essentially coronary aneurysm/ectasia remain a rare condition in adult patients with NS. Evolution is unknown and complications such as coronary artery thrombosis do not necessarily require surgical treatment.

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.

BACKGROUND: Valve replacement surgery is the definitive management strategy for patients with severe valvular disease. However, valvular conduits currently in clinical use are associated with significant limitations. Tissue-engineered (decellularized) heart valves are alternative prostheses that have demonstrated promising early results. The purpose of this systematic review and meta-analysis is to perform robust evaluation of the clinical performance of decellularized heart valves implanted in either outflow tract position, in comparison with standard tissue conduits.
METHODS: Systematic searches were conducted in the PubMed, Scopus, and Web of Science databases for articles in which outcomes between decellularized heart valves surgically implanted within either outflow tract position of human subjects and standard tissue conduits were compared. Primary endpoints included postoperative mortality and reoperation rates. Meta-analysis was performed using a random-effects model via the Mantel-Haenszel method.
RESULTS: Seventeen articles were identified, of which 16 were included in the meta-analysis. In total, 1418 patients underwent outflow tract reconstructions with decellularized heart valves and 2725 patients received standard tissue conduits. Decellularized heart valves were produced from human pulmonary valves and implanted within the right ventricular outflow tract in all cases. Lower postoperative mortality (4.7% vs. 6.1%; RR 0.94, 95% CI: 0.60-1.47; P = 0.77) and reoperation rates (4.8% vs. 7.4%; RR 0.55, 95% CI: 0.36-0.84; P = 0.0057) were observed in patients with decellularized heart valves, although only reoperation rates were statistically significant. There was no statistically significant heterogeneity between the analyzed articles (I2 = 31%, P = 0.13 and I2 = 33%, P = 0.10 respectively).
CONCLUSIONS: Decellularized heart valves implanted within the right ventricular outflow tract have demonstrated significantly lower reoperation rates when compared to standard tissue conduits. However, in order to allow for more accurate conclusions about the clinical performance of decellularized heart valves to be made, there need to be more high-quality studies with greater consistency in the reporting of clinical outcomes.

BACKGROUND: Pulmonary endarteritis is a rare clinical phenomenon with congenital heart that can potentially lead to major complications.
METHODS: We report a 47-year-old man with pulmonary endarteritis. This patient presented with hypertension, chest pain and a previous history of pulmonary valve disease during childhood. Also, eight-months prior, he was hospitalized with dyspnea (Functional Class III), cough, phlegm, and night sweats without fever. Echocardiographic diagnosis in the first transtransthoracic echocardiography (TTE) was intense pulmonary valve stenosis (PVS) an, thus, the pulmonary valve vegetation and PVS, established by transesophageal echocardiography (TEE). He was referred for surgery after 1 weeks of intravenous antibiotic therapy for removal of the vegetation.
CONCLUSIONS: Finally he was asymptomatic at 3-months of follow-up and was clinically in good condition. Therefore, the detection of infective endocarditis of the lung valve must not lengthy be prolonged.

We herein describe, for the first time, the fetal presentation of a case of ventricular inversion, restrictive ventricular septal defect, pulmonary stenosis, hypertensive left ventricle and double outlet right ventricle at 34 weeks of gestational age. Postnatal echocardiography confirmed the prenatal diagnosis. The patient was subsequently successfully palliated with a left ventricle to pulmonary artery conduit. This report illustrates the importance of detailed fetal echocardiography to ensure appropriate delivery and neonatal management, and to optimize outcome.