Sildenafil Increases Systemic Saturation and Reduces Pulmonary Artery Pressure in Patients with Failing Fontan Physiology

Objective. The purpose of this study was to investigate the effect of sildenafil in patients with failing Fontan physiology. Design. A retrospective chart review was performed to compare history and available data in patients with Fontan circulations before and after starting sildenafil. The paired and unpaired Student's t‐tests were used for statistical analyses. Patients. Six patients at our institution with Fontan physiology, persistent symptoms of cyanosis or effusion, and poor hemodynamics as measured in the catheterization laboratory were placed on sildenafil. One patient was not included in the analysis because of insufficient length of treatment. All patients had symptoms of failing Fontan hemodynamics with either persistent cyanosis or effusions. In this group, the mean pulmonary artery pressure was greater than 15 mm Hg (17.4 ± 1.5 mm Hg) with mean estimated pulmonary vascular resistance of 3.5 ± 1.0 Wood units × m² prior to starting sildenafil. Results. Sildenafil significantly increased the systemic arterial oxyhemoglobin saturation in this group (82.8 ± 7.3% pre‐treatment vs. 91.0 ± 5.5% post‐treatment, P = .017). In the four out of five patients who have had follow‐up catheterizations, there was a significant decrease in pulmonary artery pressure (17.4 ± 1.5 mm Hg pre‐treatment vs. 13.8 ± 2.1 mm Hg post‐treatment, P = .018) and in estimated pulmonary vascular resistance pre‐ and post‐sildenafil treatment (3.5 ± 1.0 Wood units × m² pre‐treatment vs. 2.0 ± 0.4 Wood units × m² post‐treatment, P = .031). Conclusions. Sildenafil may be a useful adjunct to therapy in patients with failing Fontan physiology likely through its function as a pulmonary vasodilator.     

Total Anomalous Pulmonary Venous Connection in an Adult: Comprehensive Multimodality Evaluation

An unusual case of total anomalous pulmonary venous connection surviving to adulthood without surgical correction is presented. Transthoracic echocardiography first led to this diagnosis and magnetic resonance imaging refined the anatomic diagnosis leading to successful surgical correction.     

Unusual Case of Left Partial Anomalous Pulmonary Venous Connection with Successful Surgical Management

We describe a case of left-sided partial anomalous pulmonary venous connection diagnosed in the setting of a respiratory illness affecting the right lung, which presumably elevated pulmonary pressures and worsened right-to-left shunting. The anatomical configuration appears to be highly unusual in this case, with separate supracardiac connections of the left upper pulmonary vein (LUPV) to a vertical vein (draining to the innominate vein) and the left lower pulmonary vein to the azygos vein. Successful surgical repair was undertaken with direct anastomosis of the LUPV to the left atrial appendage and creation of an intra-superior vena cava baffle to redirect azygos vein flow to the left atrium.     

Clinical Efficacy of Phosphodiesterase‐5 Inhibitor Tadalafil in Eisenmenger Syndrome—A Randomized,Placebo‐controlled,Double‐blind Crossover Study

Objectives. In a randomized double‐blind crossover trial, we compared the efficacy of phosphodiesterase‐5 (PDE‐5) inhibitor tadalafil with placebo in patients of Eisenmenger Syndrome (ES). The primary end point was the change in 6‐minute walk test distance (6 MWD). Secondary end points were the effect of the drug on systemic oxygen saturation (SO₂), pulmonary vascular resistance (PVR), systemic vascular resistance (SVR), effective pulmonary blood flow (EPBF), and World Health Organization (WHO) functional class. Background. ES is a disorder with limited treatment options. Uncontrolled studies have shown PDE‐5 inhibitors to be beneficial in patients of ES. Methods. Twenty‐eight symptomatic adult patients of ES with weight ≥30 kg in WHO class II and III were enrolled. Patients were given 40 mg of tadalafil or matching placebo for 6 weeks followed by crossover to the other drug after a washout period of 2 weeks. Assessment of WHO class, exercise capacity by 6 MWD, and various hemodynamic parameters by cardiac catheterization was done at baseline, after 6 weeks and at the end of the study. Results. All patients completed the study. There was significant increase in 6 MWD following drug administration compared with baseline (404.18 ± 69.54 m vs. 357.75 ± 73.25 m, P < .001). Compared with placebo, tadalafil produced significant decrease in PVR (?7.32 ± 1.58, P < .001), resulting in significant increase in EPBF (0.12 ± 0.05, P= .03), SO₂ % (1.72 ± 0.58, P= .007), and WHO functional class (1.96 ± 0.18 vs. 2.14 ± 0.44, P= .025), with no significant change in SVR (P= NS). Conclusion. In this first short‐term placebo‐controlled trial of tadalafil in patients of ES, the drug was well tolerated and significantly improved exercise capacity, functional class, SO₂, and pulmonary hemodynamics. http://www.clinicaltrial.gov/ct2/show/NCT01200732?term=NCT01200732&rank=1     

42例婴幼儿完全性肺静脉异位引流的治疗体会

目的:总结婴幼儿完全性肺静脉异位引流的早期诊断和治疗经验。方法:1995年3月至2005年3月,我院共完成42例完全性肺静脉异位引流的手术治疗,男33例,女9例,年龄18天～3岁,平均(4.3±3.2)月,体重3.2～11kg,平均(5.4±1.2)kg。心上型27例,心内型10例,心下型3例,混合型2例。所有患者均由超声心动图确诊,11例行心导管检查,3例心下型行磁共振成像检查。均在全麻低温体外循环下行矫治术。6例心上型采用左心房顶部入路。结果:死亡4例,死亡率9.52%(3例心上型围术期死亡,1例心内型术后1年因肺静脉回流梗阻死亡)。3例心下型无死亡。心律失常7例中,6例为心上型双心房横切口,1例为心内型。随访39例,随访时间1月～10年,存活者心功能明显改善。结论:完全性肺静脉异位引流可由超声心动图确诊,必要时辅以心导管检查及磁共振成像检查。新生儿及婴儿期手术可获得良好效果。心上法矫治心上型可减少心律失常的发生率。     

Protein‐losing Enteropathy after the Total Cavopulmonary Connection: Impact of Intravenous Immunoglobulin

Objective. Despite multiple treatment modalities, protein‐losing enteropathy remains a serious complication to Fontan‐type operations. Observations suggest inflammation to be involved in the pathogenesis of this condition, and immunomodulating treatment with high‐dose intravenous immunoglobulin may modify the condition positively. Patients. Four patients with protein‐losing enteropathy occurring after the total cavopulmonary connection, presenting with edema, hypoalbuminemia, and hypogammaglobulinemia, received intravenous immunoglobulin replacement therapy. Interventions. Standard replacement dose (1 g/kg) was used with intervals between infusions adjusted according to albumin and gamma globulin levels. Treatment periods ranged from 1 year to 5.3 years. Results. Intravenous immunoglobulin treatment was associated with significant increase in plasma albumin and to some extent in immunoglobulin G levels, as well as resolution of edema and the children started to thrive normally. During treatment, no serious infections or serious side effects were seen. Additional follow‐up intervals ranged from 2 years to 2.8 years, during which only one episode of clinical relapse was registered and treated. Conclusions. We find the increase in albumin level and the resolution of protein‐losing enteropathy symptoms after treatment with intravenous immunoglobulin of particular interest considering this serious complication to Fontan‐type operations.     

Total Anomalous Pulmonary Venous Drainage to Coronary Sinus Combined with Left‐sided Obstructive Lesions: A Previously Unreported Association

We present a very rare cardiac lesion where we found a combination of abnormal pulmonary venous drainage and left‐sided obstructive lesions. This case gives new insight into the complex malformation of the heart, raising questions about cardiac embryology and timing of interfering events. Here we describe the lesion for the first time and review the related literature. To the best of our knowledge, this combination has never been reported.     

Lowering Pulmonary Wedge Pressure after Heart Transplant: Pulmonary Compliance and Resistance Effect

Background

Right ventricular (RV) afterload is an important risk factor for post-heart transplantation (HTx) mortality, and it results from the interaction between pulmonary vascular resistance (PVR) and pulmonary compliance (CPA). Their product, the RC time, is believed to be constant. An exception is observed in pulmonary hypertension because of elevated left ventricular (LV) filling pressures.

Objective

Using HTx as a model for chronic lowering of LV filling pressures, our aim was to assess the variations in RV afterload components after transplantation.

Methods

We retrospectively studied 159 patients with right heart catheterization before and after HTx. The effect of Htx on hemodynamic variables was assessed.

Results

Most of the patients were male (76%), and the mean age was 53 ± 12 years. HTx had a significant effect on the hemodynamics, with normalization of the LV and RV filling pressures and a significant increase in cardiac output and heart rate (HR). The PVR decreased by 56% and CPA increased by 86%. The RC time did not change significantly, instead of increasing secondary to pulmonary wedge pressure (PWP) normalization after HTx as expected. The expected increase in RC time with PWP lowering was offset by the increase in HR (because of autonomic denervation of the heart). This effect was independent from the decrease of PWP.

Conclusions

The RC time remained unchanged after HTx, notwithstanding the fact that pulmonary capillary wedge pressure significantly decreased. An increased HR may have an important effect on RC time and RV afterload. Studying these interactions may be of value to the assessment of HTx candidates and explaining early RV failure after HTx.     

Prognostic Value of Pulmonary Vascular Resistance by Magnetic Resonance in Systolic Heart Failure

Background

Pulmonary hypertension is associated with poor prognosis in heart failure. However, non-invasive diagnosis is still challenging in clinical practice.

Objective

We sought to assess the prognostic utility of non-invasive estimation of pulmonary vascular resistances (PVR) by cardiovascular magnetic resonance to predict adverse cardiovascular outcomes in heart failure with reduced ejection fraction (HFrEF).

Methods

Prospective registry of patients with left ventricular ejection fraction (LVEF) < 40% and recently admitted for decompensated heart failure during three years. PVRwere calculated based on right ventricular ejection fraction and average velocity of the pulmonary artery estimated during cardiac magnetic resonance. Readmission for heart failure and all-cause mortality were considered as adverse events at follow-up.

Results

105 patients (average LVEF 26.0 ±7.7%, ischemic etiology 43%) were included. Patients with adverse events at long-term follow-up had higher values of PVR (6.93 ± 1.9 vs. 4.6 ± 1.7estimated Wood Units (eWu), p < 0.001). In multivariate Cox regression analysis, PVR ≥ 5 eWu(cutoff value according to ROC curve) was independently associated with increased risk of adverse events at 9 months follow-up (HR2.98; 95% CI 1.12-7.88; p < 0.03).

Conclusions

In patients with HFrEF, the presence of PVR ≥ 5.0 Wu is associated with significantly worse clinical outcome at follow-up. Non-invasive estimation of PVR by cardiac magnetic resonance might be useful for risk stratification in HFrEF, irrespective of etiology, presence of late gadolinium enhancement or LVEF.     

Repair of an Anomalous Left Coronary Artery from the Pulmonary Artery in a Premature Neonate

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. It is typically diagnosed at a few months of age. The infant presents with cardiac failure secondary to left ventricular dysfunction from the coronary steal that develops as pulmonary vascular resistance (PVR) falls. With the diagnosis made, surgical repair is typically performed expeditiously in order to try and restore left ventricular perfusion and stop the ongoing coronary steal. We present an unusual case of a preterm infant undergoing a routine echocardiogram, who was incidentally found to have an ALCAPA. Management strategies between neonatology and cardiology may differ in this setting. In this case, elevated PVR helps to preserve myocardial perfusion prior to surgical repair. Therefore, common neonatal management strategies typically utilized for respiratory distress, elevated PVR, and cardiac dysfunction may have potential for detrimental effects on myocardial perfusion in this specific lesion. This case also emphasizes the importance of identifying the coronary origins as a routine part of a complete pediatric echocardiogram. It also presents a rarely encountered problem: when to repair an ALCAPA in an as yet asymptomatic patient. We believe this to be the youngest and smallest patient reported with the diagnosis and successful surgical correction of an ALCAPA.     

Catheter‐guided Open Ligation of a Pulmonary Artery to Left Atrium Connection

Congenital malformations causing mild cyanosis can be overlooked. A large intrapulmonary right pulmonary artery to left atrium connection was the unusual etiology of arterial oxygen desaturation and erythrocytosis in an asymptomatic 30‐year‐old patient. The shunt was not possible to detect at echocardiography. It was closed via a median sternotomy without the aid of cardiopulmonary bypass. A novel technique was to use an angiographic catheter inserted through the wide shunt into the left atrium before the operation to securely identify it at the surgical dissection.     

Age and Method‐dependent Variability of Predicted Oxygen Consumption in Congenital Heart Disease

Background and Purpose. There has been a lot of debate about the use of predicted oxygen consumption to calculate pulmonary vascular resistance using the Fick principle. We therefore comparatively analyzed predicted oxygen consumption in infants and children in specific age groups, using different methods (formulas), as an attempt to better understand the usefulness and limitations of predictions. Methods and Results. Four models (LaFarge & Miettinen, Bergstra et al., Lindahl, and Lundell et al.) were used to predict oxygen consumption in 200 acyanotic patients with congenital cardiac defects aged 0–2.0, >2.0–4.0, >4.0–6.0, and >6.0–8.75 years (median 2.04 years). Significant differences were observed between the age groups (P < .001) and between the methods (P < .001), not related to diagnoses. Differences between methods were more impressive in the first age group (P < .01). In patients aged 0–2.0 years, the lowest values of oxygen consumption (corresponding to the highest estimates of pulmonary vascular resistance) were obtained with the method of Lindahl; above this age, any method except that of Lundell et al. Conclusions. Although measuring oxygen consumption is always preferable, a rational use of predictions, using different methods, may be of help in situations where measurements are definitely not possible.     

Left‐sided Partial Anomalous Pulmonary Venous Communication in a Patient with Turner's Syndrome

Congenital defects of the aortic valve and thoracic aorta are well recognised in patients with Turner's syndrome. Anomalous pulmonary venous connections that were previously considered rare in patients with Turner's syndrome have been detected in up to one in ten patients with the advent of cardiac magnetic resonance imaging. We describe an instance of partial anomalous pulmonary venous drainage in a patient with Turner's syndrome, discuss the clinical indicators of these anomalies, and briefly mention the management options in such patients.     

A congenitally “poor” pulmonary artery is a major reason for exclusion from Fontan operation

Summary We reviewed the clinical records of 185 patients who were possible candidates for Fontan operation. We did this to clarify whether all patients with suitable anomalies ultimately underwent a Fontan operation. Of the 152 patients with decreased pulmonary blood flow, 38 (26%) did not fulfill the criteria for Fontan operation, and 17 of the 33 patients (52%) with increased pulmonary blood flow (P<0.01) were excluded as candidates. Of 48 non-candidate survivors, 19 had high pulmonary artery (PA) pressure or resistance and small PA (which we term “poor PA”), 17 had pulmonary hypertension, 6 had a markedly distorted PA, and 6 had severe ventricular dysfunction. A significant proportion of possible candidates with a suitable anomaly ultimately did not undergo a Fontan operation, because of “poor PA”, a congenital condition that precluded Fontan operation.     

Successful coil embolization for Fontan‐rout to aorta communication—recovery from complication of brockenbrough procedure in Fontan patient

The Brockenbrough (BB) procedure is becoming the necessary procedure for radiofrequency ablation for supravalvular tachycardia in patients after a Fontan procedure. We experience Fontan‐rout to aorta communication caused by the BB procedure. The communication was successfully occluded using a detachable coil. This complication was rare but could still happen as an increasing necessity of the catheter ablation in patients with a Fontan procedure. Coil embolization is a potential option for recovery from this complication. © 2015 Wiley Periodicals, Inc.     

137例婴幼儿完全性肺静脉异位引流各年龄段临床特点和外科疗效分析

目的:探讨婴幼儿完全性肺静脉异位引流(TAPVC)各年龄段的临床特点和外科治疗效果.方法:2000-01至2006-12 诊断TAPVC并在我院进行手术治疗的婴幼儿(除外合并法乐四联症、右心室双出口、完全性房室通道、单心室等复杂畸形的患儿)共137例.分为≤28天新生儿组(组1,n=7)、＞28天～≤6个月组(组2,n=70)、＞6个月～≤1岁组(组3,n=26)、＞1岁～≤3岁组(组4,n=34)4个年龄组.137例患儿伴房间隔缺损119例,卵圆孔未闭18例,动脉导管未闭27例,室间隔缺损2例,三尖瓣关闭不全103例.合并重度肺动脉高压50.4%(69/137).其中≤6个月患儿(组1、组2)占71.0%(49/69).心上型49.6%(68/137),心内型37.2%(51/137).心下型1.5%(2/137),混合型11.7%(16/137).合并肺静脉狭窄18.2%(25/137),其中≤6个月患儿(组1、组2)占88.0%(22/25).结果:手术死亡率11.7%(16/137),组1和组2占87.5%(14/16).主要死亡原因为肺部感染和低心排综合征.有随访资料99例,随访率81.8%(99/121),随访期限2～86个月,平均(25.5±22.3)个月.晚期死亡9例,均为组1和组2患儿,晚期死亡率9.1%(9/99).结论:年龄＞6个月的TAPVC患儿可获得满意的外科治疗效果,而年龄≤6个月尤其是新生儿患儿病情重,合并限制性房间隔缺损、肺静脉狭窄和重度肺动脉高压比例高,往往需要急诊手术,手术及远期死亡率较高.