Alterations in cerebral blood flow in children with congestive heart failure due to ventricular septal defect

Objective: We aimed to investigate the effect of ventricular septal defect (VSD) and heart failure on cerebral blood flow (CBF) in children, whether heart failure treatment improves CBF, and if there is any relationship between CBF and serum N‐ terminal pro‐brain natriuretic peptide (NT‐proBNP) level.
Method: Forty children with VSD (13 with heart failure) aged between 1 and 36 months were studied. The control group comprised 25 healthy children in the same age group. Maximum, minimum, and mean blood flow velocities and pulsatility indices of the right and left middle cerebral arteries were assessed using transcranial Doppler ultrasonography. Measurements of CBF and serum NT‐proBNP levels were repeated in patients with heart failure a month post–heart failure treatment initiation. The groups were also compared in terms of defect diameters, cardiac structural changes, left ventricular systolic function, and findings related to pulmonary hypertension determined by echocardiography. Correlations between echocardiographic measurements and CBF parameters were analyzed.
Results: Although no significant difference was found between patient and control groups for CBF, right and left maximum CBF velocities significantly increased posttreatment in patients with heart failure (P = .04 and P = .01, respectively). Serum NT‐ proBNP levels in children with VSD associated with heart failure were significantly higher than those in children with VSD alone (P = .04) or in healthy children (P < .001). NT‐proBNP levels were negatively correlated with right and left maximum CBF velocities (r = −0.39, P = .013 and r = −0.32, P = .043, respectively).
Conclusion: Although no significant difference was found in CBF velocity among the study groups, increase in the CBF velocity post heart failure treatment and negative correlations between CBF velocity and both the VSD diameter and NT‐proBNP levels indicate that the hemodynamic status due to VSD associated with heart failure has an effect on CBF.     

Severe tricuspid regurgitation and isolated right heart failure due to thyrotoxicosis

We describe the case of a patient presented with isolated right heart failure with atrial fibrillation and severe tricuspid regurgitation due to hyperthyroidism. Treatment of the thyroid disease resulted in the disappearance of signs of right heart failure and resolution of the valve incompetence and normalization of the heart rhythm. Although thyrotoxicosis may be associated with congestive heart failure, isolated right heart failure with marked tricuspid regurgitation is rarely seen.     

From right to left heart failure: An unexpected transition

Right and left heart failure are very common clinical syndromes with close correlation. Right-sided or right ventricular heart failure usually occurs as a result of left-sided failure. We report a very rare case of transition from right heart failure due to pulmonary embolism, followed by its resolution, to left heart failure due to Tako-tsubo syndrome within 48 h of hospitalization.     

Severe right heart failure in a patient with grave's disease

This brief report presents a patient with isolated right heart failure and two rare underlying causes, hyperthy-roidism and dysplastic tricuspid valve. Repair of the tricuspid valve and treatment of the hyperthyroidism were both essential for successful treatment of the right heart failure. Most important, recrudescence of hyperthyroidism in this patient was associated with reappearance of florid right heart failure. This report provides further information about a potential linkage of hyperthyroidism and severe right heart failure.     

Nemaline Myopathy Initially Diagnosed as Right Heart Failure with Type 2 Respiratory Failure

Nemaline myopathy (NM) is a rare muscle disease with various clinical types. In some cases, NM can lead to type 2 respiratory failure and right heart failure. We herein report a patient with congenital NM with nebulin gene mutation who presented with acute right heart failure and type 2 respiratory failure due to respiratory muscle paralysis after upper respiratory tract infection, needing a permanent ventilator for assistance. However, the limb and trunk muscle strengths were within normal limits. This case showed that NM should be considered as a cause of right heart failure and type 2 respiratory failure.     

心室辅助装置在小儿原发性心肌病并发心力衰竭中的应用价值

目的:评价心室辅助装置在小儿原发性心肌病并发心力衰竭中的应用价值.方法:4例扩张型心肌病(DCM)患儿采用手术安装心室辅助装置代替并发心室行使泵功能.术后常规给予抗凝剂苯丙酸豆素和抗血小板药物阿司匹林以防止血栓事件的发生.术后每日采用毛细血管法测定INR,同时严密观察管道有无血栓形成.结果:1例6个月男婴安装左室辅助装置(LVAD);1例40个月男性患儿初安装LVAD,后因继发右心衰竭于9周后复手术安装双侧心室辅助装置(BIVD);1例13岁女性患儿和1例15岁男性患儿因全心力衰竭安装BIVD;4例目前心功能均恢复良好.其中6个月男婴已心脏移植,15岁男性患儿因心功能和心肌收缩力恢复良好,予撤机后痊愈出院.结论:对于原发性心肌病并发心力衰竭患儿,应用心室辅助装置如Berlin Heart系统进行治疗,效果良好.     

Progression of heart failure in right univentricular pacing compared to biventricular pacing

BACKGROUND: Cardiac resynchronization therapy (CRT) improves hemodynamics and symptoms of heart failure by reducing ventricular dyssynchronity. Conversely, recent studies have demonstrated that right univentricular pacing in patients with an ejection fraction below 40% aggravates heart failure. In this retrospective study, we compared progression of disease in patients with mild to moderate heart failure that were treated with a right univentricular pacing device and patients with congestive heart failure that were treated with a biventricular system. METHODS: 107 patients were included. 59 received a right ventricular pacing device and 48 a biventricular system. Patients were assessed after 1 and 6 months by NYHA class, echocardiographic parameters (EF, LVEDD) and hospitalization for heart failure. RESULTS: Hospitalization for heart failure after implantation of the devices was more frequent in patients that received a conventional pacemaker with a single lead in the right ventricle than in patients that were treated with a CRT system (12% vs. 6%, p<0.05), although heart failure was more advanced in the CRT group at baseline. Ejection fraction in the right ventricular pacing group further decreased from 43%+/-4 at baseline to 38%+/-4 after 6 months (p<0.05). Left ventricular enddiastolic diameter (LVEDD) was 51+/-7 mm and 58+/-6 mm (p<0.05) at 6 months. In the CRT group, EF was 23%+/-4 at baseline and 31%+/-7 after 6 months (p<0.05.). LVEDD improved from 56+/-4 mm before implantation to 52+/-7 mm and 6 months (p<0.05). CONCLUSION: Progression of heart failure symptoms in the right univentricular pacing group was more pronounced compared to the CRT group, despite the fact that patients assigned to the CRT group had more severe symptoms of heart failure at baseline. Biventricular pacing relieved symptoms of heart failure, whereas right univentricular pacing with subsequent conduction delay of the left ventricle further deteriorated pre-existing heart failure. Therefore, patients with an indication for pacemaker therapy because of bradycardia and co-existing mild to moderate heart failure might benefit from early implantation of a CRT system.     

Reversible Pulmonary Hypertension and Isolated Right-sided Heart Failure Associated with Hyperthyroidism

Hyperthyroidism may present with signs and symptoms related to dysfunction of a variety of organs. Cardiovascular pathology in hyperthyroidism is common. A few case reports describe isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension as the prominent cardiovascular manifestations of hyperthyroidism. Although most textbooks do not mention hyperthyroidism as a cause of pulmonary hypertension and isolated right heart failure, the literature suggests that some hyperthyroid patients may develop reversible pulmonary hypertension and isolated right heart failure. We report a case of hyperthyroidism presenting with signs and symptoms of isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension, which resolved with treatment of hyperthyroidism.     

Pulmonary Hypertension in Advanced Heart Failure

Abstract The diagnosis of pulmonary hypertension is easy during routine evaluation of patients with chronic heart failure by means of Doppler echocardiography. However, one must remember that an accurate hemodynamic characterization of the pulmonary circulation requires right heart catheterization to measure pulmonary vascular resistance and, if necessary, to test the reversibility of pulmonary hypertension. In addition, the importance of combining the right heart hemodynamic variables with a functional evaluation of the right ventricle is emphasized: in fact, the clinical impact of pulmonary hypertension in advanced heart failure patients (in terms of both exercise intolerance and prognosis) seems to be modulated by right ventricular function.     

心率变异性测定对心力衰竭患儿预后的意义

目的 观察心力衰竭患儿心力衰竭程度与心率变异性(HRV)的关系,探讨HRV对心力衰竭患儿预后的意义.方法 对46例心力衰竭患儿[心功能Ⅰ～Ⅱ级(NYHA分级)21例,Ⅲ～Ⅳ级25例]和30例健康儿童进行24 h动态心电图HRV测定.结果 (1)心力衰竭组HRV各参数均较对照组明显降低,心功能Ⅲ～Ⅳ级HRV各参数较Ⅰ～Ⅱ级明显降低(P＜0.05).(2)所有心力衰竭患儿随访(5.0±2.5)年,随访过程中死亡15例,病死率32.6%,死亡患儿HRV各参数明显降低,与存活患儿比较差异有统计学意义(P＜0.01).结论 HRV可反映心力衰竭的严重程度,对心力衰竭患儿预后具有参考价值.     

红细胞体积分布宽度与慢阻肺急性加重期患者病情严重程度的相关性

目的探讨红细胞体积分布宽度(RDW)与慢性阻塞性肺疾病急性加重(AECOPD)患者病情严重程度的相关性。方法收集我科收治的140例AECOPD患者1的临床资料,根据RDW是否增高分为RDW增高组和RDW正常组;比较CAT分值、住院时间(LOS)、脑钠肽(BNP)、CRP、白细胞计数、Pa CO2、Pa O2、呼吸衰竭及右心衰竭发生率等相关指标在各组间的差异。根据是否合并呼吸衰竭、右心衰竭分为;既无呼衰又无右心衰(A)组、单纯呼衰(B)组、单纯右心衰(C)组、既有呼衰又有右心衰(D)组,比较RDW值在各组间的差异。结果 RDW增高组CAT分值、住院时间(LOS)、脑钠肽(BNP)、Pa CO2、CRP、白细胞计数均高于RDW正常组(P0.05),Pa O2低于RDW正常组(P0.05)。RDW增高组呼吸衰竭、右心衰竭发生率显著高于RDW正常组(P0.001)。B、C、D组RDW值均显著高于A组(P0.05)。RDW值与Pa O2呈负相关,与白细胞计数、CRP、Pa CO2、BNP、CAT分值、LOS均呈正相关(P0.05)。结论 RDW或许可作为评估AECOPD患者病情严重程度的一项重要参考指标。     

Heart rate variability in severe right or left heart failure: the role of pulmonary hypertension and resistances

BACKGROUND: The decrease in heart rate variability (HRV) might be related to the hemodynamic status in heart failure. However, HRV in patients with severe isolated right heart failure has not been extensively studied. AIMS: This study compared HRV in patients with congestive heart failure (CHF) and in patients with isolated right heart failure. METHODS: Time and frequency domain analysis of HRV on 24-h ECG recording was assessed in 15 healthy subjects and in two groups of patients with severe heart failure awaiting heart or heart/lung transplantation. These were 15 patients with CHF due to idiopathic dilated cardiomyopathy (IDC) and 10 patients with isolated right heart failure due to primary pulmonary hypertension (PPH). RESULTS: Measurement of HRV were significantly decreased in both groups of patients compared with the control group. Patients with IDC had higher pulmonary capillary wedge pressure than patients with PPH (P=0.04) but lower pulmonary artery pressure and lower pulmonary vascular resistance (PVR) (P<0.0001). However, all the measurements of HRV were significantly lower in patients with IDC than in patients with PPH (range 22-77%, P<0.05 to P<0.01). None of the HRV measurements correlated with filling pressure measurements. CONCLUSIONS: The increase in pulmonary vascular resistance in heart failure is not the main causal factor behind a decrease in HRV.     

Left and right ventricular diastolic dysfunction and diastolic heart failure: does one lead to the other?

Background and Objective Diastolic dysfunction of the left ventricle is a mechanical abnormality diagnosed primarily by echocardiogram, and can be distinguished into three separate degrees based on the severity of reduction in passive compliance and active myocardial relaxation. Methods A literature search was performed for basic science studies, clinical studies and major practice guidelines on the subject of diastolic dysfunction and diastolic heart failure. Important findings were analyzed and correlated with regard to clinical relevance. Results Left ventricular diastolic dysfunction appears to compromise exercise tolerance and is believed to contribute to the pathophysiology in patients with diastolic heart failure. In the clinical setting, however, oftentimes no clear distinction is made between echocardiographically diagnosed diastolic dysfunction and diastolic heart failure, and adequate treatment recommendations are sparse and aimed to prevent worsening and progression of clinical symptoms. To date, there is a lack of high powered trials assessing the possible progression rate from echocardiographically diagnosed diastolic dysfunction to the clinical diagnosis of diastolic heart failure. Furthermore, there are no solid indices to assess the degree of severity of diastolic dysfunction or its progression. Pure right ventricular diastolic dysfunction appears to be even less understood and under-recognized, although it may play a role in the development of both right and left heart failure. Currently there are few but interesting data on the possible interaction between ventricles with diastolic dysfunction and the overall affect on the development of heart failure. Conclusions The timeline and progression of diastolic dysfunction to diastolic heart failure have not been well established and warrant further investigation.     

心力衰竭患者心脏机械运动周期时间变化的临床研究

目的应用超声组织多普勒的方法研究心力衰竭(简称心衰)患者心动周期各个间期的比例变化。方法对18例心衰患者(心衰组)及30例健康志愿者(对照组),在静息下标准四腔切面,测量左右心室基底段,同一心动周期中等容收缩期、快速射血期、减速射血期、等容舒张期、快速充盈期、舒张后期及心房收缩期,并换算成在收缩期和舒张期的比例。结果对照组左右心室收缩期和舒张期在心动周期的比例相同,心衰组左室收缩期和舒张期比例无明显变化,右室收缩期比例减小(43.29%±6.64%vs 38.98%±7.05%,P<0.05);在收缩期内,左室等容收缩期和快速射血期比例增加而慢速射血期比例减少,右室等容收缩期比例增加,快速射血期比例减少,慢速射血期比例不变;在舒张期内,左右心室等容舒张期比例均增加,右室快速充盈时间比例显著下降,左室无明显下降,左室舒张后期时间比例下降而右室增加,右室心房收缩时间比例下降而左室增加。对照组左右心室之间各个间期均有非常显著的差异,心衰患者左右心室之间的差异明显减小。结论心衰时心动周期的各个间期的分布比例与正常对照相比存在显著差异。左右心室之间各个间期的差异在正常对照非常显著,心衰时减小。     

Right heart dysfunction and failure in heart failure with preserved ejection fraction: mechanisms and management. Position statement on behalf of the Heart Failure Association of the European Society of Cardiology

There is an unmet need for effective treatment strategies to reduce morbidity and mortality in patients with heart failure with preserved ejection fraction (HFpEF). Until recently, attention in patients with HFpEF was almost exclusively focused on the left side. However, it is now increasingly recognized that right heart dysfunction is common and contributes importantly to poor prognosis in HFpEF. More insights into the development of right heart dysfunction in HFpEF may aid to our knowledge about this complex disease and may eventually lead to better treatments to improve outcomes in these patients. In this position paper from the Heart Failure Association of the European Society of Cardiology, the Committee on Heart Failure with Preserved Ejection Fraction reviews the prevalence, diagnosis, and pathophysiology of right heart dysfunction and failure in patients with HFpEF. Finally, potential treatment strategies, important knowledge gaps and future directions regarding the right side in HFpEF are discussed.     

Doppler velocimetry in superior vena cava provides useful information on the right circulatory function in patients with congestive heart failure

BACKGROUND: Although flow velocities curves recorded with pulsed-wave Doppler in systemic vein are known to provide functional data on the right circulatory function, little information is available on the relationship between right heart filling dynamics and right ventricular function. METHODS: Consecutive patients with chronic heart failure due to severe systolic left ventricular dysfunction and in sinus rhythm underwent echocardiography and right heart catheterization. In the initial part of the study, the hemodynamic correlates of different flow velocity patterns recorded into the superior vena cava were evaluated in 120 patients. The accuracy of the prediction of different right heart hemodynamic profiles by means of the different venous flow patterns was then prospectively tested in a subsequent series of 86 patients. RESULTS: The venous flow pattern was closely related to right heart hemodynamics. A normal Doppler pattern identified patients with normal right heart hemodynamics (sensitivity 86%, specificity 78%); a "predominant systolic wave" pattern identified patients with a reduced thermodilution-derived right ventricular ejection fraction (< 30%) and normal or slightly elevated right atrial pressure (< or = 8 mmHg) (sensitivity 69%, specificity 81%); a "predominant diastolic wave" pattern identified patients with a reduced right ventricular ejection fraction (< 3 0%) and elevated right atrial pressure (> 8 mmHg) (sensitivity 52%, specificity 95%). The observed and the predicted hemodynamic profiles turned out to be concordant in 80% of patients. CONCLUSIONS: The analysis of the flow velocity pattern into the superior vena cava is a useful tool to estimate the extent of the right circulatory impairment in patients with congestive heart failure.     

肺动脉高压所致右心衰竭的机制和治疗

肺动脉高压(PAH)是一种严重疾病。如不加控制,最终将导致右心衰竭。在这篇文章里,我们回顾肺动脉高压所致右心衰竭的机制研究进展。肺动脉高压所致右心衰竭分为急性右心衰竭和慢性右心衰竭。其中慢性右心衰竭又分为代偿性和失代偿性右心衰竭两个阶段。在急性右心衰竭过程中,右心室衰竭表现为急性炎症,如病灶内心肌细胞溶解,中性粒细胞和单核细胞趋向至病灶。负荷增加激活核因子-κB等信号通路。从而启动一系列的反应,如细胞因子释放等。同时内皮细胞释放血管紧张素Ⅱ(AngⅡ)可能加重这个反应。与急性右心衰竭不同,慢性右心衰竭的早期,右心室因机械牵张而激活AngⅠ、整合素和胰岛素样生长因子等信号通路,导致细胞骨架重组,增加钙释放和哺乳动物雷帕霉素靶蛋白(mTOR)通路的激活,随后蛋白质合成增加,最后表现为心室肥厚。转化生长因子β(TGF-β)信号通路在此过程也发挥很重要的作用。慢性右心衰竭的后期,心肌细胞失去代偿能力。右心室因机械牵张而激活整合素等信号通路,增加钙释放。基质金属蛋白酶(MMP)2和MMP9激活导致疤痕形成。有氧代谢向无氧代谢的转换也起重要作用。这种转换诱导一系列基因表达的改变,如葡萄糖转运蛋白1(GLUT-1);也包括酶,如丙酮酸脱氢酶磷酸化的改变和钾通道特性的改变。丙酮酸脱氢酶激酶在这个过程中起重要作用。     

右心室长轴运动的位移及速度在右心衰竭诊断中的价值

目的:评价不同超声心动图指标和组合方式在右心衰竭中的诊断价值。方法:右心衰竭组患者62例,对照组健康成人52例,应用超声诊断仪测量三尖瓣环收缩位移(TAPSE)、收缩期峰值速度(Sm)、游离壁基底段应变(BS)、中间段应变(MS)、右心室舒张末期面积(RVEDA)和收缩末期面积(RVESA),计算右心室面积变化率。用逐步判别法分析结果,建立诊断右心衰竭的判别模型。分别用TAPSE<15mm、Sm<11.5cm/s及二者串联和并联试验4种方法诊断右心衰竭。结果:与对照组比较,右心衰竭组RVEDA和RVESA增加,TAPSE、Sm、BS和MS显著降低(均P<0.01)。诊断右心衰竭的判别模型:右心衰竭F=1.166×TAPSE+1.732×Sm-14.134,非右心衰竭F=1.779×TAPSE+2.627×Sm-31.818;两者串联时诊断灵敏度为90.3%,特异度为88.5%,正确率为89.5%。结论:诊断右心衰竭时,TAPSE与Sm更具价值,两者串联临床意义更大。     

Endocrine causes of heart failure: A clinical primer for cardiologists

Heart failure (HF) may be a presenting manifestation of a few endocrine disorders and should be considered in evaluation of heart failure causes. This clinically oriented review is an attempt to highlight the protean manifestations of heart failure in endocrine diseases which could present either as acute or chronic heart failure. Acute heart failure manifests as hypertensive crisis, Takotsubo syndrome, or as tachy/brady cardiomyopathies. Chronic heart failure could masquerade with features of hyperdynamic heart failure, or hypertrophic, restrictive or dilated cardiomyopathy. Rarely constrictive features or resistant heart failure could be the presenting feature. Isolated presentation as pulmonary hypertension and right heart failure are also documented. Good history-taking and physical examination with targeted investigations will help in the timely management for reversing the pathophysiology to a significant extent by appropriated management.     

Sudden onset congestive heart failure with a continuous murmur: ruptured sinus of Valsalva aneurysm complicated by anomalous origin of the left coronary artery

Ruptured sinus of Valsalva aneurysm is an unusual cause for congestive heart failure, and anomalous coronary arteries have rarely been found in association. A 47-year-old man developed sudden onset heart failure due to a ruptured noncoronary sinus of Valsalva fistula to the right atrium. Coronary angiography revealed an anomalous left coronary artery arising from the right coronary sinus, limiting percutaneous options for repair. We review the incidence, complications, and management of sinus of Valsalva aneurysms and anomalous left coronary arteries.