Working backward: Retrograde balloon angioplasty of atretic arteries in chronic thromboembolic pulmonary hypertension

We describe a patient with chronic thromboembolic pulmonary hypertension who presented to the pediatric cardiac catheterization laboratory for peripheral pulmonary artery recanalization and angioplasty. This case report outlines serial pulmonary arterial rehabilitation via a retrograde approach through intrapulmonary collateral arterial connections.     

Congenital aortic atresia with intact ventricular septum and normal left ventricle. Diagnosis by cross-sectional echocardiography

We describe the second reported case of congenital aortic atresia with normal left ventricle and intact ventricular septum. We believe this to be the first case diagnosed by cross-sectional echocardiography.     

The Adult Congenital and Pediatric Cardiology Section: increasing the opportunities for the congenital heart disease community within the American College of Cardiology

The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College-advocacy, quality, education, and member engagement-serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date.     

安立生坦在先天性心脏病术后肺动脉高压中的治疗效果

目的:评估安立生坦在先天性心脏病术后肺动脉高压中的治疗效果。方法:我们选取2013年2月~12月70例先天性心脏病术后肺动脉高压的患者。随机分为试药组(n=35)和对照组(n=35)。试药组给予安立生坦+常规治疗,对照组仅给予常规治疗。12周后患者门诊随访,评估心功能状况、行心脏彩超评估肺动脉压力,并化验检测肝肾功能指标。结果:试药组[(38±5)mm Hg vs.(50±6)mm Hg,P0.05]和对照组[(41±6)mm Hg vs.(47±7)mm Hg,P0.05]患者术后12周肺动脉压力较术后第3天均明显降低,尽管如此,试药组肺动脉压力降低幅度明显大于对照组[(12±4)mm Hg vs.(6±3)mm Hg,P0.05]。试药组患者中30例(86%)心功能明显改善,对照组中25例(71%)心功能明显改善,两者有显著差异(P0.05)。试药组患者未见肝功能损害及贫血病例。结论:先天性心脏病术后肺动脉高压的患者使用安立生坦能够安全有效地降低肺动脉压力,改善心功能。     

先心病介入封堵治疗的新进展

先天性心脏病的发病率为8‰～12‰,传统外科手术是主要的治疗方法。随着微创介入手术的飞速发展,先心病介入封堵治疗日益成熟,现就目前几种常见先心病的介入封堵治疗的新进展做一综述。     

A Systematic Approach to Pulmonary Valve Replacement in the Current Era

Background: Pulmonary valve replacement (PVR) can be accomplished via surgical, transcatheter, or hybrid approaches. There are inherent advantages to transcatheter PVR and hybrid PVR without cardiopulmonary bypass. We review the methods and results of a standardized institutional approach to PVR. Methods: Retrospective review of all PVR cases between February 2017 and February 2020. Hybrid PVR entailed off-pump RVOT plication with percutaneous transcatheter PVR. Results: Primary transcatheter PVR was attempted in 37, hybrid PVR was performed in 11, and on-pump surgical PVR was performed in 9. Median age at PVR was 27 years (6–65). Primary transcatheter PVR was successful in 35/37 (2 converted to surgical). Standard surgical PVR was utilized for positive coronary compression testing (n = 4), stent/valve system migration (n = 2), or patient preference (n = 3). In the hybrid group mean RVOT diameter was 34 mm (32–38). Median length of stay was 1 day for transcatheter PVR, 5 for surgical, and 3 for hybrid (p = 0.02). Median follow-up was 1.5 years. Re-interventions were one balloon valve dilation in a transcatheter PVR, and one valve dilation with subsequent transcatheter valve-in-valve PVR in the surgical cohort. One hybrid patient expired 11 months post procedure. Conclusions: A systematic approach to PVR utilizing all approaches in pre-defined order of preference leads to consistent outcomes in a wide variety of anatomic configurations. Transcatheter PVR may be accomplished in the majority of patients. When necessary, hybrid off-pump RVOT plication with transcatheter PVR avoids the need for cardiopulmonary bypass.     

心脏停跳与不停跳先心病矫治术患者围术期cTnⅠ及心肌酶水平变化

目的探讨心脏停跳与不停跳先天性心脏病（先心病）矫治术对心肌的损伤程度,并寻找敏感性评价指标。方法将40例同期拟行先心病矫治术的患者随机分为对照组和观察组各20例,分别于心脏停跳与不停跳下手术,两组麻醉及体外循环（CPB）方法相同。分别于围术期检测血清心肌肌钙蛋白Ⅰ（cTnⅠ）、肌酸激酶同工酶（CK-MB）、乳酸脱氢酶（LDH）、肌酸激酶（CK）、AST、α-羟丁酸脱氢酶（HBDH）,同时观察心电图及心脏超声指标改变。结果两组围术期心电图及心脏超声指标均无明显异常,但术中、术后血清cTnⅠ及CK、CK—MB、LDH水平均明显升高,对照组显著高于观察组;其中血清cTnⅠ水平升高出现早、恢复慢（术后72h时仍显著高于术前水平）。结论心脏不停跳先心病矫治术对心肌的损伤小于心脏停跳手术;cTnⅠ是评价心脏手术围术期心肌损伤敏感的特异指标．     

Use of Implantable Cardioverter-Defibrillators in Congenital Heart Disease and Pediatric Patients: Results from the German National Registry for Congenital Heart Defects

Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to 2015, only 0.2% of the CHD patients in the national register received an ICD. Indication for ICD implantation was secondary prevention in 84 patients (78%) and primary prevention in 24 patients (22%). 23 patients (21%) of the ICD patients received appropriate ICD therapy. 7 patients (6%) received an inappropriate ICD therapy. In 23 patients (21%) device complications were documented with a high number of lead fractures and insulation defects (n = 14, 13%). Conclusion: The current study investigates the clinical uptake and use of ICD therapy based on a large national registry for CHD patients. Despite a steady increase in the number of implanted devices, ICD uptake remains relatively low, particularly for primary prevention. The data suggests a potential reluctance in utilization of device therapy in this patient cohort for primary prevention. Selecting patients in whom benefits outweigh the risks associated with lifelong ICD therapy remains challenging.     

Prenatal ultrasonic detection of truncus arteriosus with interrupted aortic arch and truncal valve regurgitation

The echocardiographic appearances in a case of persistent truncusarteriosus with aortic arch interruption and truncal valve dysplasiawas noted in a 24 week fetus. Elective termination of pregnancywas carried out and all the echocardiographic findings wereconfirmed by pathological examination suggesting the possibilityof accurate prenatal diagnosis of such lesions.     

Prenatal ultrasonic detection of truncus arteriosus with interrupted aortic arch and truncal valve regurgitation

The echocardiographic appearances in a case of persistent truncusarteriosus with aortic arch interruption and truncal valve dysplasiawas noted in a 24 week fetus. Elective termination of pregnancywas carried out and all the echocardiographic findings wereconfirmed by pathological examination suggesting the possibilityof accurate prenatal diagnosis of such lesions.     

Infective endocarditis in children with congenital heart disease: the changing spectrum, 1965--85

Thirty-five episodes of infective endocarditis in 35 childrenwith congenital heart disease, from January 1965 to December1984, were reviewed. The incidence of infective endocarditisin cyanotic congenital heart defects increased with a concomitantdecrease in the frequency of lesions with left to right shunt.The incidence of postoperative endocarditis decreased from 1:52during the first decade to 1:1033 during the second decade.The time interval between onset of symptoms and the establishmentof diagnosis and treatment shortened from 7.8 ± 3.2 weeks(mean ± SD) to 2.1±0.9 weeks. During the latterdecade, positive blood cultures were obtained in 100% of patients,as compared with 60% during the former decade (P 0.05). Echocardiographydemonstrated vegetations in 70% of the cases. Over the entiretime period, streptococcus viridans was the most frequent pathogen(46%) and staphylococcus aureus secondmostfrequent (17%). Sixpatients died, all were under two years of age and all had congestiveheart failure. Early surgical correction of the congenital heartdiseasemay offer the best form of prevention.     

Surgical treatment of anomalous left main coronary artery with an intraconal course

Objective: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course.
Methods: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2‐47). The seven oldest patients all had preoperative symptoms of exertional chest pain and one also had exertional syncope. The five youngest pa‐ tients had no preoperative symptoms. One of these patients had a hemodynamically significant ventricular septal defect and one patient was the sibling of a patient who had undergone repair of AAOCA.
Results: The 12 patients underwent surgical repair including unroofing of the myo‐ cardial bridge overlying the intraconal LMCA and a LeCompte procedure. There was no early or late mortality and there were no significant complications. All 12 patients have resumed normal, unrestricted activities.
Conclusions: Twelve patients with an anomalous LMCA and intraconal course pre‐ sented to our institution for treatment. Surgical repair was performed successfully in all 12, with resolution of symptoms in the 7 patients who were symptomatic preop‐ eratively. These results suggest that the surgical treatment is safe and efficacious in patients with an anomalous LMCA and intraconal course.     

Heart rate variability in healthy children and in those with congenital heart disease both before and after operation

There are no reports of standard measures of heart rate variability (HRV) in pediatric patients with heart disease. Time domain (standard deviation of all normal RR intervals [SDNN], standard deviation of all 5-minute mean RR intervals, average standard deviation of all 5-minute RR intervals, and frequency domain (total, low- [LF], and high-frequency [HF] power) measures of HRV were (1) obtained in 45 healthy children, (2) compared between 36 children with congenital heart disease and age-matched controls, (3) compared before and after surgery, and (4) compared between age-matched postoperative patients staying <7 days (group I, n = 16) and those staying longer (group II, n = 16). In healthy children, SDNN increased rapidly during infancy and more gradually thereafter, while the LH/HF ratio decreased until preschool age, with a later increase into adolescence. Compared with controls, preoperative patients had decreased total (53 ± 55 vs 84 ± 75 beats/min²/Hz, p = 0.01) and HF (12 ± 14 vs 29 ± 46 beats/min²/Hz, p = 0.03) power despite having similar heart rates. In the immediate postoperative period, all measures of HRV were decreased from preoperative values. Groups I and II did not differ in mean RR interval or HRV preoperatively; however, postoperatively, HRV was decreased in group II when compared with group I (SDNN 53 ± 17 vs 40 ± 14 ms, p = 0.01), although the mean RR interval remained comparable (499 ± 81 vs 481 ± 62 ms, p = 0.3). It is concluded that (1) there are significant age-related changes in HRV in healthy children, (2) preoperatively, children with congenital heart disease have reduced total and HF power when compared with healthy controls, (3) HRV is further reduced postoperatively in all patients, and (4) prolonged postoperative hospitalization is associated with a greater reduction in HRV.