A neonate with a spongy failing heart - What could it be?

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.

Low Mean Perfusion Pressure Indexed to Body Surface Area is a Powerful Predictor of Poor Outcomes After Heart Transplantation in Patients With High Pre-Transplant Venous Pressure: A Clinical Study With Physiological Insights From Mathematical Modelling of Biventricular Heart Failure.

BACKGROUND & AIM: The deleterious consequences of chronically elevated venous pressure in patients with profound right ventricular or biventricular dysfunction are well known, including renal and hepatic dysfunction, and volume overload. The only option for these patients, if they fail optimal medical treatment, is a heart transplant, as they are not candidates for left ventricular assist device therapy. Mean perfusion pressure (MPP) is important in the outcomes of critically ill patients with high venous pressure. The question arises whether MPP is important for the outcomes of heart transplants in patients with elevated pre-transplant venous pressure. Medical management of heart failure patients with reduced ejection fraction involves lowering the systemic afterload with vasodilators while awaiting a transplant. We hypothesised that when venous pressure is elevated prior to transplant, a substantial reduction in systemic arterial elastance (Ea) through vasodilation may significantly decrease MPP, resulting in compromised end-organ function and consequent unfavourable outcomes after heart transplantation. This study aims to investigate whether a low MPP serves as a risk factor for adverse outcomes in heart transplant recipients with high venous pressure. METHOD: A retrospective analysis was conducted on 250 heart transplant recipients undergoing isolated heart transplantation at a single institution from October 2012 to March 2020. Right atrial pressure (RAP) of more than 15 mmHg was considered high. Additionally, Ea calculated as the ratio of end-systolic pressure to stroke volume, and MPP calculated as the difference between mean arterial pressure and RAP were considered in our analysis. The outcomes of transplantation were measured in terms of 90-day mortality and survival up to 7 years. RESULTS: High RAP was a significant risk factor for short-term and medium-term survival if Ea was low (<2.7 mmHg/mL, the median value). This group had 39.39% in-hospital mortality compared to 14.49% for RAP<15 mmHg (p∼0.005). When Ea was high, this difference in survival was not evident: 8% for RAP<15 mmHg vs 4.8% for RAP>15 mmHg (p∼0.550). This effect was mediated through a lower MPP, and the mortality due to lower MPP increased strikingly with higher body surface area (BSA). A negative correlation was observed between MPP indexed to BSA (MPPI) and the Model for End-Stage Liver Disease score (r∼-0.3580, p<0.0001) as well as creatinine (r∼-0.3551, p<0.0001). MPPI less than 40 mmHg/m2 was associated with poorer short-term (23.2% for MPPI<40 mmHg/m2 vs 7.1% for MPPI>40 mmHg/m2, p∼0.001) and medium-term survival. The impact of high RAP and low Ea on survival was evident even on medium-term follow-up; only 30% survival at 7 years follow-up for high RAP and low Ea vs 75% for RAP<15 mmHg (p∼0.0033). CONCLUSION: The acceptable blood pressure during vasodilator therapy in patients with high RAP needs to be higher, especially in those with higher BSA. MPPI less than 40 mmHg/m2 is a risk factor for survival, in the short and medium-term, after heart transplantation.

Virtual reality-guided left ventricular assist device implantation in pediatric patient: Valuable presurgical tool.

Virtual reality (VR) is increasingly used for presurgical planning and teaching during surgery. However, VR aided presurgical planning toolbox for left ventricular assist device (LVAD) implantation is not widely available. We investigated the use of a VR environment with wearable headsets and touch controllers in simulating an implant in an 11-year-old boy. The technology played a significant role in the optimal positioning of the LVAD.

Heart transplantation for patients with single ventricle physiology.

BACKGROUND: There is a growing population of palliated and unpalliated single ventricle physiology patients for whom heart transplantation is the only treatment option available. There is a paucity of reports of heart transplantation in this challenging and growing subset of patients from our part of the world. The purpose of the article is to briefly review our experience in the subgroup and compare it with the available literature. METHODS: This was a single-institution retrospective observational study of 16 patients with single ventricle physiology who were transplanted between 2016 and 2019 and their outcomes. The study groups were divided into those with ventricular dysfunction (group 1), who fare substantially better than those with normal ventricular function (group 2) whose short-term outcomes were poorer. Worsening cyanosis, poor candidature for completion Fontan procedure due to severe atrioventricular valve regurgitation or pulmonary artery anatomy, protein-losing enteropathy, plastic bronchitis, and worsening systemic venous congestion are indications in those with normal ventricular function. RESULTS: Patients with ventricular dysfunction as the main indication had excellent early survival with no early mortality compared to 40% mortality in patients with normal ventricular function. Patients who survived to leave the hospital had however similar long-term outcomes. Two patients with protein-losing enteropathy resolved completely by one month. Normal ventricular function, pulmonary artery stenting, early Fontan failure (6 months), ascites, and need for desensitization were risk factors for early mortality. After the early acute phase of increased risk, the mortality risk plateaued off. CONCLUSION: Transplantation in patients with single ventricle and ventricular dysfunction can be offered with a good early and late outcome. There is a need to have multi-institutional and multi-disciplinary collaboration along with work in basic sciences to better understand the effects of failed Fontan physiology with normal ventricular function.

Cardiac transplantation for cardiomyopathy with constrictive pericarditis.

Constrictive pericarditis is a great mimic and has posed a diagnostic dilemma since its first description 300 years ago as "Concretio Cordis." It can mimic restrictive cardiomyopathy, endomyocardial fibrosis, and chronic liver and renal disease. This would perhaps be the first clinical report of constriction in patients undergoing cardiac transplantation. We report two distinct cases with cardiomyopathy requiring cardiac transplantation and the clinical implications of concomitant pericardial constriction. While the first case mimics a natural "cardiac support device," which addresses ventricular remodeling in heart failure by reducing the wall stress, the second case is a case in point against the use of "biological pericardial membrane-like the bovine pericardium," as a pericardial substitute.

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.

Cost analysis of 163 consecutive heart transplant operationsin an Indian setting.

OBJECTIVES: Heart transplantation has become an established procedure in India with increasing numbers being done annually. The majority of these patients are very sick with a history of multiple hospital admissions. The economic burden of such therapy is substantial and the costs are borne by the patients with very little insurance coverage. The objective of this study was to estimate the following:The average cost of a heart transplant procedure in a "real-world setting" in a private healthcare facility in India, with varying patient risk profiles.Identify the factors contributing to the wide variations in cost seen in practice.Finally, based on data thus collected, can some kind of estimation be made about the expected cost in a given patient before the operation is done? METHODS: The cost incurred by the hospital in doing 168 heart transplants during a 3-year period was analyzed. Costs were divided into direct and indirect costs. The direct costs consisted of medical consumables, laboratory investigations, radiology, costs involved in organ harvest and transport, and diet. Direct cost was 40 % of the total cost of the procedure and was used as a surrogate for total costing estimates. RESULTS: There was a wide variation in direct costs almost tenfold, ranging from INR 240,882 to 2403193 with a mean of 603755.The cost was affected by length of stay in the hospital, whether a patient survived or died and whether the patient died within 7 days of surgery or later. It was also affected by patient-specific factors like the INTERMACS status and preoperative creatinine levels. The average cost of the entire transplant procedure was INR 1459000. CONCLUSION: There is a wide variation in the cost incurred by the hospital in doing transplants dictated to a large extent by patient risk profiles. A "One package fits all model" is unrealistic. The data pertains to the cost to the hospital and not the hospital bill and is therefore relevant to any health care facility, public or private. This study provides a framework for Governmental and private insurance agencies to fix the reimbursement rates.

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.

Aortic valve dynamics using a fluid structure interaction model--The physiology of opening and closing.

Comparative study among aortic valves requires the use of an unbiased and relevant boundary condition. Pressure and flow boundary conditions used in literature are not sufficient for an unbiased analysis. We need a different boundary condition to analyze the valves in an unbiased, relevant environment. The proposed boundary condition is a combination of the pressure and flow boundary condition methods, which is chosen considering the demerits of the pressure and flow boundary conditions. In order to study the valve in its natural environment and to give a comparative analysis between different boundary conditions, a fluid-structure interaction analysis is made using the pressure and the proposed boundary conditions for a normal aortic valve. Commercial software LS-DYNA is used in all our analysis. The proposed boundary condition ensures a full opening of the valve with reduced valve regurgitation. It is found that for a very marginal raise in the ventricular pressure caused by pumping a fixed stroke volume, the cardiac output is considerably raised. The mechanics of the valve is similar between these two boundary conditions, however we observe that the importance of the root to raise the cardiac output may be overstated, considering the importance of the fully open nodule of arantius. Our proposed boundary condition delivers all the insights offered by the pressure and flow boundary conditions, along with providing an unbiased framework for the analysis of different valves and hence, more suitable for comparative analysis.

Normal aortic valves stay open much longer in systole than porcine substitutes.

OBJECTIVE: To compare the opening mechanics of porcine valve substitutes with those of a normal human aortic valve. BACKGROUND: All commercially available porcine valves are pretreated with glutaraldehyde. This study was undertaken to evaluate the consequences of such treatment on valve mechanics. METHODS: The opening mechanics of the aortic valve, especially the time taken to open fully from a closed position, and the duration for which the valve is maximally open, were compared in a normal aortic valve, a stent-mounted porcine valve, and a stentless porcine valve, using a finite element model. RESULTS: Despite a 4-fold higher gradient, stent-mounted porcine valves were slower in attaining the fully open position, and the time for which the valve was fully open was almost 25% less than a normal valve. In stentless valves, the compliant root made the initial opening mechanics similar to those of a normal valve. Once this effect was over, the effect of porcine leaflet properties took over, and there was a corresponding delay in the valve opening. CONCLUSIONS: Fixing the root with a stent and stiffening the leaflets with glutaraldehyde result in delayed valve opening and decrease the duration for which the valve is fully open, thus contributing to inferior hemodynamics.

Tetralogy of Fallot with pheochromocytoma: an unusual therapeutic challenge.

An adult with unoperated tetralogy of Fallot diagnosed simultaneously with pheochromocytoma is extremely rare. This poses obvious diagnostic and therapeutic challenges. A 29-year-old woman with these conditions was successfully operated on for both diseases in the same hospitalization. There is some interesting speculation regarding the association of pheochromocytoma with uncorrected cyanotic congenital heart disease.

Modified septosuperior approach for the repair of supracardiac total anomalous pulmonary venous return in infants.

An alternative technique for the repair of supracardiac total anomalous pulmonary venous return (TAPVR) is described. The pulmonary venous confluence (CPV) is identified below the right pulmonary artery between the aorta and superior vena cava. The atrial incisions are similar to a septosuperior approach of the mitral valve, modifying the incision on the roof of the left atrium to be parallel to the incision in the CPV. The common pulmonary vein is anastomosed to the roof of the left atrium. This approach offers optimal exposure for the repair without distorting the cardiac structures.

Severe coronary artery disease with coarctation of the aorta: role of off-pump coronary artery bypass grafting.

Severe coronary artery disease with coarctation of the aorta is an unusual and challenging clinical problem. We encountered three adults with severe coronary artery disease and tight coarctation of the aorta. Since angina was the dominant symptom in all, off-pump coronary artery bypass grafting (OPCABG) was done as a first stage. All survived the operation. After a gap of 3 weeks, coarctation repair has been done in 1 patient. We believe that OPCABG offers some unique advantages in this difficult situation.