Surgical outcomes of common arterial trunk repair beyond infancy.

Background: The aim of this study is to analyze the clinical outcomes of common arterial trunk repair beyond infancy in terms of both early- and long-term outcomes. Methods: Between January 2003 and December 2019, 56 patients underwent repair for common arterial trunk beyond infancy at our institute. Median age was 34.5 months, 51.8% were females, and 48.2% were males. Results: 48.2% were type 1, 46.4% were type 2, and 5.4% were type 3. 17.9% patients underwent direct connection technique for right ventricular outflow tract reconstruction; remaining received a conduit. The most common type of truncal valve anatomy was tricuspid (82.1%). Early mortality was 7%. Univariable analysis identified age (p = 0.003), weight (p = 0.04), duration of ventilation (p = 0.036), and pulmonary hypertensive crisis (p ≤ 0.001) as factors affecting early mortality. In our overall cohort of beyond infancy repair for common arterial trunk, at 10 years, the survival, freedom from reintervention for right ventricular outflow tract reconstruction, freedom from ≥ moderate conduit obstruction, freedom from impaired right ventricle function, and freedom from ≥ moderate truncal valve regurgitation were 76.7%, 89.7%, 74%, 88.6%, and 66.3%, respectively. Conclusion: Repair for common arterial trunk in patients presenting beyond 1 year of age is challenging; however, it can be done with satisfactory early and late outcomes in terms of mortality and reintervention.

Case Report-Right atrial myxoma with total anomalous pulmonary venous connection in neonate.

INTRODUCTION AND IMPORTANCE: Myxoma is the commonest cardiac tumor in adults but is very rare in a newborn. The majority of myxoma arises from the interatrial septum. 75% of myxomas are found in the left atrium and 20% are found in the right atrium. Myxomas can be single or multiple. Multiple myxomas are usually associated with familial syndromes. Myxomas are generally not associated with another congenital heart defect except atrial septal defect. As myxomas are rare in newborns most of the knowledge is through various case reports. CASE PRESENTATION: We report a rare case of an 8-day-old neonate presenting with cyanosis and respiratory distress. Transthoracic echocardiography was suggestive of right atrial myxoma which was arising from the atrioventricular junction on the right side of an interatrial septum. It was associated with the total anomalous pulmonary venous connection. The patient underwent successful excision of myxoma and rerouting of the common venous chamber to the left atrium with an uneventful immediate postoperative course. CLINICAL DISCUSSION: Cardiac myxomas are rare in newborns and their association with TAPVC even rare. Our patient had single atrial myxoma with TAPVC. The significance of this association is not clear. This association is mostly by chance. Probably it is the first case reported of its kind. CONCLUSION: As myxomas are very rare in neonates, it is highly possible to miss other associated structural heart defects. Diagnosis of atrial myxoma can be suggested by echocardiogram but a definite diagnosis can only be confirmed by histology.

Neonatal intensive care unit on-site surgery for congenital diaphragmatic hernia.

The present study presents the experience gained in the Newborn Intensive Care Unit (NICU) of 'Maria S. Curie' Emergency Clinical Hospital for Children in Bucharest (Romania) after performing a series of bedside surgery interventions on newborns with congenital diaphragmatic hernia (CDH). We conducted a retrospective analysis of the data for all patients operated on-site between 2011 and 2020, in terms of pre- and post-operative stability, procedures performed, complications and outcomes. An analysis of a control group was used to provide a reference to the survival rate for non-operated patients. The present study is based on data from 10 cases of newborns, surgically operated on, on average, on the fifth day of life. The main reasons for operating on-site included hemodynamical instability and the need to administer inhaled nitric oxide (iNO) and high-frequency oscillatory ventilation (HFOV). There were no unforeseen events during surgery, no immediate postoperative complications and no surgery-related mortality. One noticed drawback was the unfamiliarity of the surgery team with the new operating environment. Our experience indicates that bedside surgery improves the likelihood of survival for critically ill neonates suffering from CDH. No immediate complications were associated with this practice.

In vivo assessment of a single adenine mutation in 5'UTR of Endothelin-1 gene in paediatric cases with severe pulmonary hypertension: an observational study.

OBJECTIVE: Endothelin-1 plays an important role in the pathogenesis of severe pulmonary hypertension. The + 139 'A', adenine insertion variant in 5'UTR of edn1 gene has been reported to be associated with increased expression of Endothelin-1 in vitro. The aim of present study was to explore the association of this variant with the circulating levels of Endothelin-1 in vivo using archived DNA and plasma samples from 38 paediatric congenital heart disease (cyanotic and acyanotic) patients with severe pulmonary hypertension. RESULTS: The plasma Endothelin-1 levels were highly varied ranging from 1.63 to75.16 pg/ml. The + 139 'A' insertion variant in 5'UTR of edn1 was seen in 8 out of 38 cases with only one acyanotic sample demonstrating homozygosity of inserted 'A' allele at + 139 site (4A/4A genotype). The plasma Endothelin-1 levels in children with homozygous variant 3A/3A genotype were comparable in cyanotic and acyanotic groups. Lone 4A/4A acyanotic sample had ET-1 levels similar to the median value of ET-1 associated with 3A/3A genotype and was absent in cyanotic group presumably due to deleterious higher ET-1 levels. The discussed observations, limited by the small sample size, are suggestive of homozygous adenine insertion variant posing a risk in cyanotic babies with Severe Pulmonary Hypertension.

A huge right ventricular hydatid cyst related to tricuspid valve causing severe pulmonary hypertension.

Preserving tricuspid valve's function is the cornerstone in surgical excision of cardiac hydatid cysts present in the right side of the heart. The perfect surgical technique may not exist. However, further studies are needed to ensure the clear path.

Pre-Operative Sildenafil for Patients With Pulmonary Hypertension Undergoing Mitral Valve Surgery: A Systematic Review and Meta-Analysis.

BACKGROUND: Pulmonary hypertension is a usual complication of long-standing mitral valve disease. Perioperative pulmonary hypertension is a risk factor for right ventricular failure and is an important cause of morbidity and mortality in patients with pulmonary hypertension undergoing mitral valve surgery. Phosphodiesterase-5 inhibitors particularly sildenafil citrate have proven clinical benefit for pulmonary arterial hypertension but have shown discordant results in group 2 pulmonary hypertension patients. We sought to determine the effect of pre-operative sildenafil on the intra-operative hemodynamic parameters of these patients. METHODS: Studies were included if they satisfied the following criteria: 1) Randomized controlled trials; 2) Adult patients with pulmonary hypertension scheduled for elective mitral valve surgery; and 3) Reported data on changes in pre-, intra-, and post-operative hemodynamic parameters. Using PUBMED, Clinical Key, Science Direct, and Cochrane databases, a search for eligible studies was conducted from September 1 to December 31, 2018. The quality of each study was evaluated using the Cochrane Risk of Bias Tool. The primary outcome of interest is on the effect of pre-operative sildenafil on the improvement of intra-operative hemodynamic parameters such as systolic pulmonary artery pressure (sPAP), mean pulmonary arterial pressure, mean arterial pressure, pulmonary and systemic vascular resistances. We also investigated its effect on the post-operative mortality, length of cardiopulmonary bypass time, ventilation time, and inotrope support requirement. Review Manager 5.3 was utilized to perform analysis of random effects for continuous outcomes. RESULTS: We identified three studies involving 153 patients with pulmonary hypertension undergoing mitral valve surgery, showing that among those who received pre-operative sildenafil there is a significant decrease in intra-operative systolic pulmonary arterial pressure (mean difference -11.19 (95% confidence interval (CI), -20.23 to -2.15), P < 0.05) and post-operative sPAP (mean difference -13.67 (95% CI, - 19.56 to - 7.78), P < 0.05) without significantly affecting the mean arterial pressure (mean difference 1.94 (95% CI, -5.49 to 9.37), P < 0.05). The systemic and pulmonary vascular resistances were not affected as well. CONCLUSIONS: Administration of pre-operative sildenafil to patients with pulmonary hypertension undergoing mitral valve surgery decreases intra-operative and post-operative systolic pulmonary arterial pressure without significantly affecting other systemic hemodynamic parameters.

Egg and banana sign of severe pulmonary arterial hypertension.

The egg and banana sign can be seen on chest computed tomography (CT) in patients with severe pulmonary arterial hypertension (PAH). It is identified by the presence of the pulmonary artery (PA) lateral to the aortic arch with the aortic arch being described as the banana and the PA as the egg.

Síndrome de Eisenmenger em um gato / Eisenmenger syndrome in cat

A 6-month-old female, 1.0kg, uncastrated female Persian cat was brought to the Veterinary Hospital at State University of Ceara, with a history of dyspnea, prostration, hyporexia and progressive weight loss for a month. On physical examination, systolic cardiac murmur, cyanosis and dyspnea were detected. Unfortunately, the cat died during oxygen therapy. Necropsy examination revealed an increase in cardiac silhouette and ventricular septal defect of 2cm in diameter. Macroscopically the lungs were collapsed, with absent and diffusely reddish blackish crepitus, and the liver with blackish red coalescent multifocal areas, interspersed with lighter areas and lobular pattern with irregular brownish multifocal areas intercepted by brownish areas. Thus, the necropsy results together with the history and physical examination of the animal confirmed the diagnosis of Eisenmenger Syndrome, becoming the report of the first case, in a cat, in Brazil.(AU)

Pulmonary hypertensive crisis and its efficient management. A Case report and literature review.

Perioperative anaesthetic management of patients with pulmonary hypertensive crisis is complex and challenging because of limited window period and treatment options. This case report describes the successful management of a patient with severe valvular heart disease superimposed by severe pulmonary hypertension, who developed pulmonary hypertensive crisis soon after induction of general anaesthesia for cardiac surgery. Pulmonary hypertensive crisis is not a usual phenomenon in these patients, but if it occurs it can be fatal. Anticipatory care of the patient to prevent crisis is the mainstay of treatment, otherwise it adds additional morbidity to such patients. Fortunately our patient survived the crisis without causing any additional morbidity during his hospital stay.

Epidural anesthesia: A safe option for cesarean section in parturient with severe pulmonary hypertension.

Rheumatic heart disease is the most common cardiac disease complicating pregnancy in developing countries. Heart disease accounts for 15% pregnancy-related mortality. In the presence of maternal heart disease, the circulatory changes of pregnancy may result in exacerbation of the hemodynamic perturbations due to complex cardiac valvular lesions leading to decompensation or death of mother or fetus. Determining the ideal anesthetic technique for cesarean section in the presence of complex cardiac conditions remains a much debated topic. General anesthesia is associated with a further increase in pulmonary pressure in response to laryngoscopy and intubation along with myocardial depression by anesthetic agents. Neuraxial blockade may lead to decrease in systemic vascular resistance and cardiac output. We report the successful anesthetic management of a parturient suffering from rheumatic heart disease with multivalvular lesions resulting in severe pulmonary hypertension under epidural anesthesia with good maternal and neonatal outcome. Successful management requires vigilant perioperative monitoring and thorough knowledge of the hemodynamics of complex cardiac valvular disease.

Extracorporeal membrane oxygenation 2016: an update.

The use of extracorporeal membrane oxygenation (ECMO) is an important issue for intensivists, critical care nurses, surgeons, cardiologists, and many others. There has been a continued increase in the number of centres performing ECMO. This review examines novel applications and recent trends in the use of ECMO over the last 2 years. These include ECMO to facilitate the safe use of other treatments, changing the timing of initiation, newer equipment and better biocompatibility, and the ability of ECMO programs to essentially choose which cluster of potential complications they are prepared to accept. ECMO continues to evolve, diversify in its applications, and improve in safety.

Challenges and opportunities in treating inflammation associated with pulmonary hypertension.

INTRODUCTION: Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension. AREAS COVERED: Historically the first inflammatory cell identified in the pulmonary vascular lesions was the mast cell. T and B lymphocytes, as well as macrophages, are present in and around the pulmonary arterioles and many patients have elevated blood levels of interleukin 1 and 6; some patients show elevated levels of leukotriene B4. An overlap between collagen-vascular disease-associated pulmonary arterial hypertension (PAH) and idiopathic PAH exists, yet only a few studies have been designed that evaluate the effect of anti-inflammatory treatments. Here we review the pertinent data that connect PAH and inflammation/autoimmune dysregulation and evaluate experimental models of severe PAH with an emphasis on the Sugen/athymic rat model of severe PAH. Expert commentary: We postulate that there are several inflammatory phenotypes and predict that there will be several anti-inflammatory treatment strategies for severe PAH.

Anomalous origin of left coronary artery from pulmonary artery associated with pulmonary hypertension.

This is a report on a 10-year-old child with anomalous origin of left coronary artery (LCA) from pulmonary artery (ALCAPA), severe pulmonary hypertension (PH), old myocardial infarction and poor intercoronary collateralization. It discusses the echocardiographic pitfalls in this particular setting and introduces a new echocardiographic view (posterior pulmonary cusp view) for visualization of the anomalous origin of LCA from the posterior pulmonary cusp (PC) in patients with ALCAPA from the PC of the pulmonary artery. We describe three echocardiographic pitfalls that can mislead the echocardiographer and two helpful hints that guide the clinician to the correct diagnosis. The survival of this child shows that limited size of left ventricular myocardial infarction and severe mitral regurgitation in early infancy can result in a life-saving pulmonary hypertension which preserves viability and function of left ventricle despite lack of intercoronary collateral arteries. After one year follow-up, she is doing well on medical treatment.

Feasibility of real-time three-dimensional echocardiography for the assessment of distorted biventricular systolic function in patients with cor pulmonale.

BACKGROUND: This study was to investigate the feasibility of real-time 3-dimensional echocardiography (RT3DE) for the analysis of biventricular ejection fractions and volume measurements in patients with cor pulmonale and the correlations of RT3DE results with 64-slice multi-detector cardiac computed tomography (64-MDCT) results. METHODS: This study included a total of 22 patients (59.3 ± 16.6 years of age; 10 males and 12 females) who showed flattening or reverse curvature of the interventricular septum and severe pulmonary hypertension [mean right ventricular (RV) systolic pressure = 66.8 ± 19.7 mmHg] on 2-dimensional transthoracic echocardiography due to cor pulmonale. Biventricular end-diastolic and end-systolic volumes were measured by RT3DE and 64-MDCT. The severity of D-shaped deformation was evaluated by using left ventricular (LV) eccentricity index (ratio of diameters parallel/perpendicular to the interventricular septum on parasternal short axis images of the papillary muscle level). RESULTS: There were moderate correlations between biventricular volumes measured by RT3DE and 64-MDCT except for LV end-systolic volume (59.8 ± 17.1 vs. 73.2 ± 20.2 mL, r = 0.652, p = 0.001 for LV end-diastolic volume; 30.6 ± 9.1 vs. 30.8 ± 12.5 mL, r = 0.361, p = 0.099 for LV end-systolic volume; 110.1 ± 42.9 vs. 171.1 ± 55.3 mL, r = 0.545, p = 0.009 for RV end-diastolic volume; and 80.9 ± 35.0 vs. 128.7 ± 45.1 mL, r = 0.549, p = 0.005 for RV end-systolic volume respectively). CONCLUSION: This study suggests that RT3DE may be a modest method for measuring distorted biventricular end-systolic and end-diastolic volumes in patients with cor pulmonale.