Congenital Hyperlipidemia in Infants for Congenital Cardiac Surgery.

Infants with concurrent severe hypertriglyceridemia and complex congenital heart disease are a rare occurrence and can have life-threatening consequences when undergoing surgical intervention. This case series outlines two instances involving infants undergoing total anomalous pulmonary venous connection repair and surgical closure of a ventricular septal defect. The study explores troubleshooting the effects of hypertriglyceridemia on perioperative outcomes.

Combined Warden and Mustard procedure for late presenting transposition with anomalous pulmonary venous connections and regressed left ventricle.

The presence of systemic and pulmonary venous anomalies in late presenting transposition of great arteries (TGA) may make standard atrial switch procedures difficult, necessitating modification in the surgical procedure. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein with TGA and regressed left ventricle, where a combination of Warden procedure and Mustard procedure was used.

Arterial switch for double-outlet left ventricle - Diagnostic and surgical considerations.

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both the aorta and pulmonary artery arise completely or predominantly from the left ventricle. DOLV is a spectrum and can be classified depending on the position of the ventricular septal defect (VSD) relative to the great vessels, the relationship of the great vessels, and the presence or absence of pulmonary or aortic outflow obstruction. In the absence of tricuspid atresia or hypoplastic right ventricle, two ventricle repair is the preferred surgical treatment. We report a 31-day-old, 2.1 kg neonate with DOLV, subaortic VSD who underwent a successful arterial switch with VSD closure.

Submitral Aneurysm Presenting as Pulsating Epigastric Mass-Diagnostic and Surgical Considerations.

Submitral aneurysm (SMA) is a rare anomaly that typically occurs adjacent to the posterior leaflet of the mitral valve. We report the case of a ten-year-old female child presenting with SMA with anteroinferior extension, differing from the more typical posterior and lateral extension. Successful surgical management is described.

Cell-free DNA in the surveillance of heart transplant rejection.

BACKGROUND: Circulating cell-free deoxyribonucleic acid (cfDNA) is promptly materializing as a highly useful tool for the surveillance of solid-organ transplant rejection. Donor-specific fraction (DF) cfDNA is a potential marker of selective donor organ injury. It is emerging as a promising analytical target in the near future. The aim of this systematic review is to throw light on the importance of cfDNA and future perspective in detecting acute rejection in heart transplantation. METHODS: An exhaustive search was carried out for this review article on the basis of literature available including scientific databases of PubMed, Embase, and ClinicalTrials.gov. The search engines were systematically explored using the search terms "cell free DNA," "Heart transplant," and "Rejection" from inception until August 2020, and narrative analysis was accomplished. Majority of the studies described endomyocardial biopsy-proven acute rejection as reference standard. RESULTS: After initial screening of 331 articles, 11 studies were included and discussed in detail in the present review article. Majority of the studies showed prospective designs. A firm correlation was noted between acute rejection (identified on endomyocardial biopsy) and cfDNA levels by most of the studies. CONCLUSIONS: cfDNA is a promising tool to replace repeated biopsies to detect rejection. The development in the area of digital droplet polymerase chain reaction and massive parallel sequencing, along with the overall reduction in cost of sequencing with its automation, has helped establish its role in the transplant population.

Use of ivabradine for treatment of junctional ectopic tachycardia in post congenital heart surgery.

Cardiac surgeries especially involving crux of the heart as performed in tetralogy of Fallot (TOF) and pulmonary stenosis are mainly responsible for junctional ectopic tachycardia (JET). Diversified antiarrhythmic agents have been used in an impressive way to treat JET but showed suboptimal efficacy and varied associated adverse effects. But, ivabradine has proved as final crusader for its treatment. We report our initial experience of 4 cases in last 6 months with ivabradine in the management of postoperative JET. Encouraged by various reports and our increasing experience with ivabradine in heart failure population, we have moved to ivabradine as the first drug of choice for postoperative JET. Bradycardia was the only significant adverse effect in our series. The availability of atrial and ventricular pacing wires or at least transvenous temporary pacing should be ensured before starting ivabradine.

Hypoxia due to unusual right to left shunt after arterial switch surgery.

Hypoxia can occur after repair of transposition of great arteries. The most common cause of right to left shunt after arterial switch surgery is related to increased right ventricular pressures and persistent neonatal pulmonary arterial hypertension. We report a case of TGA repair causing right to left shunt with normal right ventricular pressures. Persistence of Eustachian valve with patent foramen ovale (PFO) is the unusual cause of hypoxia and desaturation. The patient was successfully managed by excision of Eustachian valve and closure of PFO.

Review of investigational drugs for coronavirus disease 2019.

In December 2019, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) became evident in Wuhan, China, and then spread rapidly worldwide. Numerous drugs and vaccines are under clinical trial pipeline for investigation against coronavirus disease 2019 (COVID-19) infection. The aim of this systematic review was to discuss about investigational new as well as repurposed drugs currently under trial for COVID-19 infection. An exhaustive search was carried out for this review article including scientific databases of PubMed, Embase, ClinicalTrials.gov, WHO International Clinical Trials Registry Platform, Web of Science, ScienceDirect, ProQuest, Google Scholar, and Scopus search engines using keywords of "Coronavirus," "COVID-19," "MERS-CoV," "MERS," "SARS-CoV-2," and "SARS-CoV-1" and "Solidarity trial" and their Persian-equivalent keywords from inception until May 2020. After screening the 296 articles searched from different databases (PubMed = 97 and other search engines = 199), 52 articles were included in the final systematic review. It was found that the World Health Organization introduced a Solidarity international clinical trial to discover an effectual treatment of COVID-19. Based on established in vitro and in vivo activity against different strains of coronaviruses, four repurposed drugs - remdesivir, lopinavir/ ritonavir combination, lopinavir/ritonavir with beta-1a, chloroquine, and hydroxychloroquine - were considered for clinical trial against COVID-19. A number of other drugs and vaccines are under clinical trial pipeline for investigation against COVID-19 infection. Despite multitude of treatment options available, treatment of choice is still not well established. Moreover, optimum supportive care and monitoring of seriously ill patients is the need of the hour.

Clinical profiles and risk factors for early and medium-term mortality following heart transplantation in a pediatric population: A single-center experience.

AIMS AND OBJECTIVES: There is a paucity of data regarding the outcomes of Heart transplantation in children from the Indian subcontinent. The data of patients under the age of 18 undergoing an isolated heart transplantation was analyzed for patient clinical profiles and risk factors for early and medium-term mortality. Hospital mortality was defined as death within 90 days of transplantation and medium-term survival as follow up of up to 6 years. MATERIALS AND METHODS: A total of 97 patients operated between March 2014 and October 2019 were included in this study. Data was collected about their INTERMACS status, pulmonary vascular resistance, donor heart ischemic times, donor age, donor to recipient weight ratio and creatinine levels. RESULTS: The age range was from 1 to 18 with a mean of 10.6 ± 4.6 years. 67 % patients were in INTERMACS category 3 or less.12 children were on mechanical circulatory support at the time of transplant. The 90 day survival was 89 %. The risk factors for hospital mortality was lower INTERMACS category (odd's ratio 0.2143, P = 0.026), elevated creatinine (odd's ratio 5.42, P = 0.076) and elevated right atrial pressure (odd's ratio 1.19, P = 0.015). Ischemic time, pulmonary vascular resistance (PVR) and PVR index (PVRI) had no effect on 90 day survival. Kaplan Meier estimates for 5 year survival was 73 %. The medium term survival was affected by INTERMACS category (Hazard ratio 0.7, P = .078), donor age > 25 (Hazard ratio 1.6, P = 0.26) and raised serum creatinine values.(Hazard ratio 2.7, P = 0.012). All the survivors are in good functional class. CONCLUSIONS: Excellent outcomes are possible after heart transplantation in a pediatric population even in a resource constrained environment of a developing economy. More efforts are needed to promote pediatric organ donation and patients need to be referred in better INTERMACS category for optimal outcomes.

Insight into Noninvasive Radiological Modalities to Detect Heart Transplant Rejection.

Purpose Patients with end-stage heart failure who remain symptomatic even with exemplary medical and device therapy are treated with heart transplantation. Multitudes of endeavor have been contrived during the last decennium in the field of noninvasive tests to rule out heart transplant rejection (HTR). In spite of having supportive literature, noninvasive imaging techniques lack acceptable documentation of clinical robustness, and endomyocardial biopsy (EMB) still remains the gold standard. The aim of this review is to shed light on the existing noninvasive radiological modalities to detect rejection among heart transplant recipients. Methods A comprehensive search was conducted for this review article on the basis of literature available including scientific databases of PubMed, Embase, and Google Scholar, using keywords of "Heart transplantation," "Acute allograft rejection," "Arrhythmias," "Echocardiography," "Speckle tracking echocardiography," and "Cardiac magnetic resonance imaging" from inception until September 2020. Results After preliminary screening of the databases, details regarding existent noninvasive radiological modalities to detect HTR were gathered and compiled in this review article. Currently, deformation imaging using speckle tracking and T2 time using cardiac magnetic resonance imaging can serve as screening tools based on which further invasive investigations can be planned. Standardization of blood-based and imaging modalities as screening and possible diagnostic tools for rejection would have obvious clinical and financial benefits in the care of growing number of post heart transplant recipients in our country. Conclusion Diagnosis of allograft rejection in heart transplant recipients through noninvasive techniques is demanding. To unravel the potential of noninvasive radiological modalities that can serve as a standard-of-care test, a prospective multicentric study randomizing noninvasive modality as first strategy versus current EMB-based gold standard of care is the need of the hour.

Transplantation for chemotherapy-induced cardiomyopathy-case series and review of current practice.

Multimodality therapies have improved the survival after tumors like Ewing's sarcoma and breast cancer. However, cardiotoxicity following chemotherapy remains an important concern. We report a case series of four patients who presented to our heart team with severe dilated cardiomyopathy along with biventricular involvement. Two of the patients were females and had breast cancer for which they were treated with trastuzumab and had developed chemotherapy induced cardiomyopathy (CCMP). The other two patients were males who had Ewing's sarcoma who developed CCMP following treatment with doxorubicin.

Role of fibrillin-1 genetic mutations and polymorphism in aortic dilatation in patients undergoing intracardiac repair of tetralogy of Fallot.

OBJECTIVE: The purposes of this study were to identify the occurrence of fibrillin-1 gene polymorphisms or mutations in exons 24 to 28 and to identify the relationship between "DNA sequence variants" and aortic dilatation in the presence of abnormal aortic histopathology and other variables in patients undergoing intracardiac repair of tetralogy of Fallot. METHODS: Operatively excised full-thickness aortic wall tissue and 5 to 10-mL venous blood samples from 74 consecutive patients undergoing intracardiac repair of tetralogy of Fallot were studied. Histopathologic evaluation was done by light microscopy. Polymerase chain reaction amplification of fibrillin-1 gene was carried out for 5 exons (24-28), and amplified products were subjected to single-strand conformation polymorphism analysis to identify sequence alterations, if any. Logistic regression analysis was done to identify the relationship between patients with and without "exonic DNA variants" with other risk factors causing aortic dilatation. RESULTS: Sixteen aortic tissue specimens (21.6%) were indicated as histologically normal and used as controls. Of 51 patients with dilated aorta, 48 (94.1%) exhibited histologic abnormalities. The incidences of significant lamellar loss, abnormal histopathology, and fibrillin-1 "DNA sequence variants" in tetralogy of Fallot with dilated aorta were 78.4%, 96.1%, and 50.9%, respectively. The risk of aortic dilatation was 8.83 (1.94-13.99) times greater in patients with histologically abnormal aorta and 8.11 (1.93-34.04) times greater in patients with fibrillin-1 "exonic DNA variants." CONCLUSION: Our findings indicate the existence of "exonic DNA variants" involving the fibrillin-1 gene in 1 or more exons (exon 24-28). The "DNA sequence variants" are more pronounced in patients with tetralogy of Fallot and dilated aorta in the presence of abnormal aortic histopathology.

Myocardial injury in coronary artery bypass grafting: on-pump versus off-pump comparison by measuring high-sensitivity C-reactive protein, cardiac troponin I, heart-type fatty acid-binding protein, creatine kinase-MB, and myoglobin release.

OBJECTIVES: We sought to investigate the release pattern of different cardiac biomarkers (high-sensitivity C-reactive protein, cardiac troponin I, heart-type fatty acid-binding protein, creatine kinase-MB, and myoglobin) and to establish the diagnostic discrimination limits of each marker protein to evaluate perioperative myocardial injury in patients undergoing coronary artery bypass grafting with or without cardiopulmonary bypass. METHODS: Fifty patients were randomly assigned to on-pump or off-pump coronary artery bypass grafting. All cardiac biomarkers were measured in serial venous blood samples drawn before heparinization in both groups and after aortic unclamping at 1, 2, 4, 8, 24, 48, and 72 hours in the on-pump group. In the off-pump group samples were taken after the last distal anastomosis and at same time intervals as in the on-pump group. RESULTS: The total amount of heart-type fatty acid-binding protein, cardiac troponin I, and high-sensitivity C-reactive protein released was significantly higher in the on-pump group than in the off-pump group. Receiver operating characteristic curve analysis of cardiac biomarkers indicated cardiac troponin I and heart-type fatty acid-binding protein as the superior diagnostic discriminators of myocardial injury, with an optimal cutoff value of greater than 0.92 ng/mL (area under the curve, 0.95 [95% CI, 0.88-1.00]; sensitivity, 92%; specificity, 92%; likelihood ratio [+], 11.50) and greater than 6.8 ng/mL (area under the curve, 0.94 [95% CI, 0.88-1.00]; sensitivity, 88%; specificity, 88%; likelihood ratio [+], 7.33), respectively. Logistic regression analysis revealed that patients with increased cardiac troponin I levels of greater than 0.92 ng/mL and heart-type fatty acid-binding protein levels of greater than 6.8 ng/mL were at 132.25 (95% confidence interval, 17.14-1020.49) times and 53.77 (95% confidence interval, 9.76-296.12) times higher risk of myocardial injury after on-pump coronary artery bypass grafting. CONCLUSIONS: Off-pump coronary artery bypass grafting provides better myocardial protection than on-pump coronary artery bypass grafting. Cardiac troponin I and heart-type fatty acid-binding protein, but not high-sensitivity C-reactive protein, served as superior diagnostic discriminators of perioperative myocardial damage after on-pump coronary artery bypass grafting.

An alternative technique for transfer of anomalous left coronary artery from the pulmonary trunk in children and adults using autogenous aortic and pulmonary arterial flaps.

BACKGROUND: Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous artery arises from the right posterior pulmonary sinus. We report a new technique for re-implantation using combined autogenous aortic and pulmonary arterial flaps in situations when a direct connection was not possible. PATIENTS AND METHODS: We have treated 4 patients, aged 3 months, 6 months, 18 months, and 27 years respectively, who presented with anomalous origin of the left coronary artery from the left posterior pulmonary sinus. We used our proposed technique for transfer because lack of coronary arterial length, diminished vessel elasticity, and extensive collaterals around the pulmonary sinuses prevented direct attachment. RESULTS: There was no early or late death. Postoperatively, all patients are in functional class I, with good biventricular function at a median follow-up of 74 months, with a range from 9 to 96 months. Postoperative coronary angiography in our 4th patient showed good arterial flow, without any distortion. CONCLUSIONS: The potential benefits of this modification of the trapdoor technique are excellent operative exposure, use of autogenous and viable tissue capable of further growth, avoidance of injury to the aortic and pulmonary valvar apparatus and production of obstruction within the right ventricular outflow tract, complete elimination of use of pericardium for augmentation of the neo-aortic tube, achievement of the anastomosis with correct angling and length, and the possibility of implantation in all patients, including adults, regardless of the distance from the aorta or the coronary arterial configuration.

Novel techniques for tumor thrombectomy for renal cell carcinoma with intraatrial tumor thrombus.

BACKGROUND: Radical nephrectomy with tumor thrombectomy in patients with renal cell carcinoma and level I to III thrombus extension is directly associated with an improved prognosis. However, radical surgery in patients with level IV thrombus extension is associated with high perioperative mortality, even if long-term survival is possible. In this report, we describe an alternative technique of vena caval and intraatrial tumor thrombectomy to decrease perioperative mortality and morbidity. METHODS: A cohort of 6 patients aged 46, 50, 53, 56, 54, and 52 years underwent radical nephrectomy with tumor thrombectomy from the vena cava and right atrium under mild hypothermic cardiopulmonary bypass and intermittent cross-clamping of the supraceliac abdominal aorta. Intraatrial tumor thrombectomy was performed on a beating, perfused heart in 4 patients and a hypothermic, cardioplegia-perfused heart in 2 patients. RESULTS: There were no early or late deaths. The aortic cross-clamp time was 12 and 15 minutes for patients 5 and 6, respectively. The cumulative hepatic and renal ischemic time was 16 minutes (range, 14 to 22 minutes) at 32 degrees C. The mean cardiopulmonary bypass time was 53.3 +/- 8.9 minutes (range, 40 to 65 minutes). At a mean follow-up of 43 +/- 24.6 months (range, 10 to 70 months), all patients are active and remain disease-free. CONCLUSIONS: We conclude that radical nephrectomy and tumor thrombectomy in patients with level IV thrombi can be safely performed with cardiopulmonary bypass, mild hypothermia. and intermittent supraceliac abdominal aortic occlusion, avoiding potential hematologic, hepatic, renal, neurologic, and septic complications associated with circulatory arrest.

Intra-aortic balloon counterpulsation in a patient with the failing Fontan circulation.

We report a 23-year-old patient undergoing functionally univentricular repair who developed cardiac failure due primarily to systemic ventricular dysfunction. The failing Fontan circulation was successfully re-established using intra-aortic balloon counterpulsation. Aortic counterpulsation facilitates recovery of systemic ventricular function, and appears to be a reasonable alternative in select instances of cardiac failure in patients with the Fontan circulation.

Serial radionuclide angiographic assessment of left ventricular ejection fraction and regional wall motion after mitral valve replacement in patients with rheumatic disease.

BACKGROUND: This study was designed to prospectively investigate the effects of total or partial chordal-sparing and chordal-nonsparing mitral valve replacement (MVR) procedures on regional and global ventricular function in a rheumatic population using sequential multiple-gated acquisition scan. METHODS: One hundred five consecutive patients undergoing different techniques of chordal preservation procedures during MVR are included in this prospective study. Fifteen patients had complete excision of the chordopapillary apparatus (group 1), 34 had total chordal preservation (group 2), and 56 had preservation of the posterior chordopapillary apparatus (group 3). Radionuclide-derived left ventricular ejection fraction (LVEF) and regional wall motion (RWM) studies on 99 survivors were performed preoperatively, at discharge, at 1 year, and at 2 years. RESULTS: At discharge, there was a decline in LVEF in all the 3 groups. Statistically significant improvement in ejection fraction occurred in the chordal preservation groups (2 and 3) (P < .05) as compared with the nonchordal group immediately and late postoperatively. Only the total chordal group (2) demonstrated complete recovery of LVEF by 2 years postoperatively. Significant and persistent RWM abnormalities were noted in both the nonchordal and posterior chordal groups. CONCLUSIONS: We conclude that complete retention of the chordopapillary apparatus during MVR provides superior results compared with nonchordal and partial chordal preservation in terms of preservation of LVEF and reduced incidence of abnormal postoperative RWM.