Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease

Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis.

Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications.

Expert opinion: PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.     

Massive obliterative right heart thrombus presenting with near‐syncope

Intracardiac thrombi are commonly encountered as a complication of a recent myocardial infarction, heart failure, atrial fibrillation, or intracardiac devices. The prevalence of atrial thrombi in the absence of these risk factors is not well‐described, but seems to be low. We present a case of a 51‐year‐old man with a massive mobile thrombus in the right heart extending through the tricuspid valve, diagnosed on echocardiography after presenting with a presyncopal episode.     

Short‐term results in infants with multiple left heart obstructive lesions

Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death.
Results: For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/− septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow‐up to three years of age.
Conclusions: This study showed excellent short‐term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.     

Liguzinediol对阿霉素诱导的慢性心衰大鼠心功能的影响

目的 通过阿霉素（Dox）复制大鼠慢性心力衰竭（CHF）模型，观察Liguzinediol对CHF大鼠心功能的影响。方法 通过血流动力学观察Liguzinediol对Dox（腹腔注射，2 mg/kg）诱导的CHF大鼠左心室内压最大上升/下降速率(±dp/dt_max)、左心室内压(LVSP)、动脉收缩压(ASP)、动脉舒张压(ADP)和心率(HR)的变化；观察Liguzinediol对血清一氧化氮（NO）、一氧化氮合成酶（NOS）、超氧化物歧化酶（SOD）、肿瘤坏死因子-α（TNF-α）和白细胞介素-6（IL-6）以及血浆中丙二醛（MDA）的影响。结果 Liguzinediol能增加LVSP、+dp/dt_max、ASP、ADP、AP、HR，降低-dp/dt_max(P<0.05~0.01)；降低NO、iNOS以及MDA的浓度，同时增强了SOD的活性(P<0.05~0.01)；抑制IL-6和TNF-α的生成(P<0.05~0.01)。结论 Liguzinediol可明显改善Dox诱导的CHF大鼠血流动力学指标，减少模型大鼠炎症因子的释放以及抑制氧自由基的生成。      

冠状动脉粥样硬化性心脏病与缺血性脑卒中相关性研究进展

冠状动脉粥样硬化性心脏病(冠心病)和缺血性脑卒中是动脉粥样硬化性疾病中最主要的2种疾病,二者可以单独或合并发生,是引起死亡和功能缺陷的重要病因。2种疾病存在一些共同的危险因素,在疾病发生上有一定的联系。明确二者之间的相关性有助于动脉粥样硬化性疾病的预防及治疗,减轻疾病不良预后引起的负担,提高国民生存质量。目前有诸多研究致力于探究二者在发病机制、疾病进展、预后以及影像学表现上的关联,其中某些研究结果之间存在差异。现将相关最新研究结果综述如下。     

Cognitive impairment associated with increased mortality rate in patients with heart failure: A systematic review and meta-analysis

BackgroundRecent systematic review and meta-analysis showed that the prevalence of cognitive impairment was significantly increased in patients with heart failure (HF) when compared to the general population. However, the effect of cognitive impairment on cardiovascular outcome in this population is still unclear. We performed a systematic review and meta-analysis to assess whether cognitive impairment associated with worse outcome in patients with HF.MethodsWe comprehensively searched the databases of MEDLINE and EMBASE from inception to October 2018. Included studies were published cohort (prospective or retrospective) or randomized control trials that evaluate the effect of cognitive impairment mortality in HF patients. Data from each study were combined using the random-effects, generic inverse variance method of DerSimonian and Laird to calculate pooled hazard ratios (HR) and 95% confidence intervals (CI).ResultsEight studies were included in the analysis involving 3318 participants (951 participants had cognitive impairment). In a random-effects model, our analysis demonstrated that cognitive impairment significantly increased the risk of mortality in HF patients (pooled HR = 1.64, 95% CI = 1.42–1.88, I² = 0.0%, p < 0.001).ConclusionOur systematic review and meta-analysis showed that the presence of cognitive impairment is strongly associated with an increased mortality risk in the HF population. Further research is needed to explore the pathophysiology of this association.     

Is stenting for atherosclerotic renal stenosis an effective technique?

IntroductionOne of the treatments for renal artery stenosis is endovascular intervention, but its effectiveness is controversial. The present study aims to analyze the experience of a working group in the endovascular treatment of selected patients with severe obstructive atherosclerotic lesions of the renal arteries, and to characterize early and late results.MethodsThis is a retrospective study of symptomatic patients with atherosclerotic renal artery stenosis who underwent endoluminal therapy between May 12, 1999 and March 12, 2015 at two institutions. Statistical analysis was performed using the PASW Statistics program.ResultsA total of 99 patients were treated, mean age 66 years and 76.8% male. The mean degree of stenosis measured by renal Doppler echocardiography was 83% and 64.6% were ostial lesions. Mean preoperative creatinine level was higher than the postoperative mean: 1.3 vs. 1.2 mg/dl (p=0.014). The number of antihypertensive drugs in the preoperative period was higher than in the postoperative period: 2.0 vs. 1.3 (p=0.001). The mean follow-up was 40 months (0-164). The mean peak systolic velocity over time in the postoperative period was 77 cm/s (40-250). The restenosis rate was 8%, and 30-day mortality was 0%.ConclusionsThe results demonstrated that the endovascular technique has a beneficial effect on blood pressure and renal function in selected patients, and is a safe technique associated with a high rate of technical success and few complications.     

10-Year Heart Failure Outcomes From Nurse-Driven Clinics in Rural Sub-Saharan Africa

Nurse-led delivery care models have the potential to address the significant burden of heart failure in sub-Saharan Africa. Starting in 2006, the Rwandan Ministry of Health, supported by Inshuti Mu Buzima (Partners In Health–Rwanda), decentralized heart failure diagnosis and care delivery in the context of advanced nurse-led integrated noncommunicable clinics at rural district hospitals. Here, the authors describe the first medium-term survival outcomes from the district level in rural sub-Saharan Africa based on their 10-year experience providing care in rural Rwanda. Kaplan-Meier methods were used to determine median time to event for: 1) composite event of known death from any cause, lost to follow-up, or transfer to estimate worst-case mortality; and 2) known death only. Five-year event-free rates were 41.7% for the composite outcome and 64.3% for known death. While death rates are encouraging, efforts to reduce loss to follow-up are needed.     

Prenatal diagnosis of caudal regression with heterotaxy syndrome: “A mermaid with a broken heart”

Caudal regression syndrome (CRS) is a rare congenital malformation with varying degrees of early gestational developmental failure. It is characterized by agenesis of the sacrum and lumbar spine, with lower limb neurological deficit and accompanying deformities of the pelvis, lower extremities, genitourinary, and gastrointestinal systems. We report a case of CRS associated with rare complex congenital heart defect, that is, heterotaxy syndrome, diagnosed prenatally.