Transplantation of heterozygous familial hypercholesterolemia living donor liver resulting in early myocardial infarction: a possible dangerous link.

Living donor liver transplantation (LDLT) is a lifesaving procedure that is often curative for several liver diseases. Familial hypercholesterolemia (FH) is a metabolic disease that results from an autosomal dominant mutation in the low-density lipoprotein receptor; yet, young patients with FH can live years without detection. Herein, we report a case of a patient who developed early myocardial infarction (MI) after having a transplant from a donor with undetected heterozygous FH. This was a 67-year-old female with non-alcoholic steatohepatitis-related liver cirrhosis, free from coronary artery disease, who underwent LDLT from her daughter, a 45-year-old female with no past medical history. One year post-transplant, she presented with an acute MI with a large atherosclerotic burden. Genetic analysis confirmed heterozygous FH in the donor but not in the recipient. This case emphasizes the importance of incorporating a thorough clinical history and lipid profile into pre-transplant testing for both the recipient and donor, as well as aggressive lipid-lowering therapy post-transplantation to avoid cardiovascular complications.

Real-world analysis of integration of sacubitril/valsartan into clinical practice in Saudi Arabia.

Despite the demonstrated advantages of angiotensin receptor/neprilysin inhibitors in the management of heart failure, the pivotal Angiotensin-Neprilysin Inhibition versus Enalapril in Heart Failure (PARADIGM-HF) trial, which explored this class of medications, did not include individuals from Saudi Arabia. Recognizing that different nations and ethnic groups may exhibit unique characteristics, this study aimed to compare the demographics and outcomes of patients in Saudi Arabia who received sacubitril/valsartan (Sac/Val) with those enrolled in the PARADIGM-HF trial. In this retrospective, multicenter cohort study, we included all adult patients diagnosed with heart failure with reduced ejection fraction (HFrEF) within a tertiary healthcare system in Saudi Arabia between January 2018 and December 2021 and were initiated on Sac/Val. The primary objective was to compare the patient characteristics of those initiating Sac/Val treatment with the participants in the PARADIGM-HF trial. The secondary endpoints included the initiation setting, dose initiation, and titration, as well as alterations in B-type natriuretic peptide and ejection fraction at the 6-month mark. Furthermore, we reported the hospitalization and mortality event rates at the 12-month time point. The study included 400 patients with HFrEF receiving Sac/Val. Compared with the PARADIGM-HF trial, the cohort had a younger mean age and a higher prevalence of diabetes mellitus. SAC/VAL was prescribed as the initial therapy for 34% of the patients, while the remaining participants were initially treated with either an angiotensin-converting enzyme inhibitor or an angiotensin receptor blocker before transitioning to Sac/Val. Approximately 75% of patients were initiated on 100 mg Sac/Val twice daily, and 90% initiated therapy in the inpatient setting. The mean ejection fraction significantly improved from 26.5 ±â€…8.4% to 30.5 ±â€…6.4% at 6 months (P < .001), while the median B-type natriuretic peptide level change was not significant (P = .39). Our study revealed notable disparities in the baseline characteristics of patients with HFrEF compared with those in the PARADIGM-HF trial. These findings offer valuable real-world insights into the prescription patterns and outcomes of Sac/Val in patients with HFrEF in Saudi Arabia, an aspect not previously represented in the PARADIGM-HF study.

2023 National Heart Center/Saudi Heart Association Focused Update of the 2019 Saudi Heart Association Guidelines for the Management of Heart Failure.

Background: The burden of cardiovascular diseases is undeniable in local populations, who have high mortality rates and a young age of disease onset. A systematic review of emerging evidence and update of the Saudi Heart Association (SHA) 2019 heart failure (HF) guidelines was therefore undertaken. Methodology: A panel of expert cardiologists reviewed recommendations of the 2019 guidelines following the Saudi Heart Association methodology for guideline recommendations. When needed, the panel provided updated and new recommendations endorsed by the national heart council that are appropriate for clinical practice and local resources in Saudi Arabia. Recommendations and conclusion: The focused update describes the appropriate use of clinical assessment as well as invasive and non-invasive modalities for the classification and diagnosis of HF. The prevention of HF was emphasized by expanding on both primary and secondary prevention approaches. Pharmacological treatment of HF was supplemented with recommendations on newer therapies, such as SGLT-2 inhibitors. Recommendations were also provided on the management of patients with cardiovascular and non-cardiovascular co-morbidities, with a focus on cardio-oncology and pregnancy. Updated clinical algorithms were included in support of HF management in both the acute and chronic settings. The implementation of this focused update on HF management in clinical practice is expected to lead to improved patient outcomes by providing evidence-based comprehensive guidance for practitioners in Saudi Arabia.

Spontaneous Coronary Artery Dissection in a Transplanted Heart.

We report the case of a 37-year-old man who presented with shortness of breath 1 year post heart transplantation. He was receiving tacrolimus, methylprednisolone, and mycophenolate. An angiogram showed spontaneous coronary artery dissection involving the left anterior descending artery. Percutaneous coronary intervention was performed successfully, with stent placement and return of flow. (Level of Difficulty: Advanced.).

Predicting Survival After VA-ECMO for Refractory Cardiogenic Shock: Validating the SAVE Score.

BACKGROUND: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is used increasingly to support patients who are in cardiogenic shock. Due to the risk of complications, prediction models may aid in identifying patients who would benefit most from VA-ECMO. One such model is the Survival After Veno-Arterial Extracorporeal Membrane Oxygenation (SAVE) score. Therefore, we wanted to validate the utility of the SAVE score in a contemporary cohort of adult patients. METHODS: Retrospective data were extracted from electronic health records of 120 patients with cardiogenic shock supported with VA-ECMO between 2011 and 2018. The SAVE score was calculated for each patient to predict survival to hospital discharge. We assessed the SAVE score calibration by comparing predicted vs observed survival at discharge. We assessed discrimination with the area under the receiver operating curve using logistic regression. RESULTS: A total of 45% of patients survived to hospital discharge. Survivors had a significantly higher mean SAVE score (-9.3 ± 4.1 in survivors vs -13.1 ± 4.4, respectively; P = 0.001). SAVE score discrimination was adequate (c = 0.77; 95% confidence interval 0.69-0.86; P < 0.001). SAVE score calibration was limited, as observed survival rates for risk classes II-V were higher in our cohort (II: 67% vs 58%; III: 78% vs 42%; IV: 61% vs 30%; and V: 29% vs 18%). CONCLUSIONS: The SAVE score underestimates survival in a contemporary North American cohort of adult patients with cardiogenic shock. Its inaccurate performance could lead to denying ECMO support to patients deemed to be too high risk. Further studies are needed to validate additional predictive models for patients requiring VA-ECMO.

CONTEXTE: L'oxygénation extracorporelle veino-artérielle (ECMO-VA) est de plus en plus utilisée comme assistance pour les patients qui sont en choc cardiogène. En raison du risque de complications, des modèles de prédiction peuvent aider à déterminer quels patients bénéficieraient le plus d'une ECMO-VA. Le score SAVE (Survival After Veno-Arterial Extracorporeal Membrane Oxygenation) est un modèle de ce genre. Par conséquent, nous voulions valider l'utilité du score SAVE dans une cohorte contemporaine de patients adultes. MÉTHODOLOGIE: Des données rétrospectives ont été extraites de dossiers médicaux électroniques de 120 patients atteints d'un choc cardiogène ayant reçu une ECMO-VA entre 2011 et 2018. Le score SAVE a été calculé pour chaque patient afin de prédire la survie au congé de l'hôpital. Nous avons évalué la calibration du score SAVE en comparant la survie prédite au moment du congé et la survie observée. Nous avons évalué la discrimination par l'aire sous la courbe opérationnelle chez le receveur en faisant appel à la régression logistique. RÉSULTATS: Au total, 45 % des patients ont survécu au congé de l'hôpital. Les survivants affichaient un score SAVE moyen considérablement plus élevé (­9,3 ± 4,1 chez les survivants vs ­13,1 ± 4,4; p = 0,001). La discrimination par le score SAVE était adéquate (c = 0,77; intervalle de confiance à 95 % : 0,69 à 0,86; p < 0,001). La calibration du score SAVE était limitée, car les taux pour les classes de risque II à V étaient plus élevés dans notre cohorte (II : 67 % vs 58 %; III : 78 % vs 42 %; IV : 61 % vs 30 %; et V : 29 % vs 18 %). CONCLUSIONS: Le score SAVE sous-estime la survie dans une cohorte nord-américaine contemporaine de patients adultes atteints d'un choc cardiogène. L'inexactitude de ses résultats pourrait faire en sorte qu'une assistance par ECMO serait refusée à des patients jugés comme présentant un risque élevé. D'autres études sont nécessaires pour valider d'autres modèles de prédiction pour les patients ayant besoin d'une ECMO-VA.

Risk evaluation using gene expression screening to monitor for acute cellular rejection in heart transplant recipients.

BACKGROUND: Gene expression profiling (GEP) was developed for non-invasive surveillance of acute cellular rejection. Despite its widespread use, there has been a paucity in outcome data for patients managed with GEP outside of clinical trials. METHODS: The Outcomes AlloMap Registry (OAR) is an observational, prospective, multicenter study including patients aged ≥ 15 years and ≥ 55 days post-cardiac transplant. Primary outcome was death and a composite outcome of hemodynamically significant rejection, graft dysfunction, retransplantation, or death. Secondary outcomes included readmission rates and development of coronary allograft vasculopathy and malignancies. RESULTS: The study included 1,504 patients, who were predominantly Caucasian (69%), male (74%), and aged 54.1 ± 12.9 years. The prevalence of moderate to severe acute cellular rejection (≥2R) was 2.0% from 2 to 6 months and 2.2% after 6 months. In the OAR there was no association between higher GEP scores and coronary allograft vasculopathy (p = 0.25), cancer (p = 0.16), or non-cytomegalovirus infection (p = 0.10). Survival at 1, 2, and 5 years post-transplant was 99%, 98%, and 94%, respectively. The composite outcome occurred in 103 patients during the follow-up period. GEP scores in dual-organ recipients (heart-kidney and heart-liver) were comparable to heart-alone recipients. CONCLUSIONS: This registry comprises the largest contemporary cohort of patients undergoing GEP for surveillance. Among patients selected for GEP surveillance, survival is excellent, and rates of acute rejection, graft dysfunction, readmission, and death are low.

Implantable cardiac defibrillator and mortality in non-ischaemic cardiomyopathy: an updated meta-analysis.

OBJECTIVES: The benefit of implantable cardiac defibrillator (ICD) in symptomatic patients with systolic dysfunction and non-ischaemic cardiomyopathy remains controversial. We conducted a systematic review and meta-analysis to determine the effect of ICD in patients with non-ischaemic cardiomyopathy on (1) all-cause mortality, (2) cardiovascular mortality and (3) sudden cardiac death. METHODS: We searched citations in meta-analyses published until 2012, and in MEDLINE, Embase, PubMed and Cochrane databases from 2012 to October 2016. We included randomised controlled trials (RCTs) evaluating the effect of ICD therapy on all-cause and cardiovascular mortality and sudden cardiac death in patients with non-ischaemic cardiomyopathy. Independent reviewers evaluated study eligibility, abstracted data and assessed risk of bias in duplicate. We used random-effect models to meta-analyse relative risks (RR) and hazard ratios (HR) across studies, the Grades of Recommendation, Assessment, Development, and Evaluation system to quantify absolute effects and quality of evidence, and I2 to evaluate heterogeneity. RESULTS: We identified six RCTs including 1715 patients experiencing 421 deaths. ICD therapy was associated with reduced overall mortality (HR 0.78, 95% CI 0.66 to 0.92, I2 = 0%, risk difference 4.7%, high quality), cardiovascular mortality (RR 0.77, 95% CI 0.60 to 0.98, I2 = 39%, risk difference 3.3%, high quality) and sudden cardiac death (RR 0.45, 95% CI 0.29 to 0.70, I2 = 0%, risk difference 4.1%, high quality). The benefit of ICD was not influenced by the use of amiodarone in the comparison group, the duration of follow-up, by use of β-blockers and ACE inhibitors/angiotensin receptor blocker or cardiac resynchronisation therapy. CONCLUSION: Primary prevention ICD therapy reduces all-cause and cardiovascular mortality and sudden cardiac death in patients with non-ischaemic cardiomyopathy.

Early pneumopericardium after heart transplantation.

A 60-year-old woman with a history of dilated cardiomyopathy underwent heart transplantation. One month post discharge, she presented to clinic with low-grade fever and productive cough. Her chest radiograph showed air-fluid levels in the pericardial silhouette. Transthoracic echocardiogram showed a large complex pericardial collection with no evidence of cardiac tamponade. The patient was urgently taken to the operating room for exploration. A large "egg-shaped" mass in the pericardium measuring 10 × 12 cm with gaseous material was aspirated. As the posterior wall of the mass was firmly adhered to the right atrium, the capsule was incompletely excised. We present the case of a potentially life-threatening complication post transplantation that required surgical debridement and life-long antibiotic suppressive therapy. To our knowledge, this is the first report of purulent pericardial collection caused by Enterobacter cancerogenous. Further research is required to better understand the biology of this microorganism and the role it may play as a pathogen in immunocompromised patients following solid organ transplantation.

Percutaneous left atrial decompression in adults with refractory cardiogenic shock supported with veno-arterial extracorporeal membrane oxygenation.

BACKGROUND AND AIM OF THE STUDY: Left ventricular (LV) distention, a recognized complication in patients supported with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) for refractory cardiogenic shock, can lead to pulmonary edema, increased myocardial oxygen consumption, and LV thrombus formation. Atrial septostomy was examined as a management strategy for LV distension. METHODS: Of 72 patients supported with VA-ECMO, seven patients underwent atrial septostomy through a trans-septal approach. The primary indication for atrial septostomy was refractory pulmonary edema. RESULTS: The mean time from ECMO initiation to LA decompression was 1.3 days (range 0-2 days). There was a 100% procedural success rate with improvement in pulmonary edema. Five patients survived to discharge with one patient exhibiting recovery of biventricular function, two patients were transplanted, one patient was decannulated, and one patient was transitioned to long-term durable ventricular assist device. Two patients died, one from multi-organ failure and one with severe anoxic brain injury. CONCLUSION: Atrial septostomy is an effective method of LV decompression that can be performed safely with a high success rate.