法洛氏四联症根治术后肺动脉瓣置换术研究进展

法洛氏四联症根治术后肺动脉瓣置换术手术时间,目前尚存在争议。指征包括显著的肺动脉瓣反流合并重度右心室扩大（右心室舒张末容积指数〉150ml／m^2或〉2倍左心室舒张末容积指数）、持续性心房扑动／心房颤动或持续性室性心动过速及QRS间期〉180ms等。术后右心室功能迅速改善,表现为右心室容量、质量的减少,QRS间期缩短等。反流的肺动脉瓣常用同种移植物、生物瓣、机械瓣代替。经导管人工肺动脉瓣置换手术及镶嵌治疗可作为外科手术的补充治疗。     

法乐四联症合并肺动脉瓣缺如的矫治

目的 :探讨法乐四联症合并肺动脉瓣缺如 (TOF/ APV)的外科治疗方法。　　方法 :TOF/ APV患者 5例 ,临床上无明显呼吸道压迫症状 ,均在体外循环下矫治。用同种肺动脉 (4例用带单瓣主肺动脉片 ,1例用带瓣主肺动脉管道 )结合自体心包片重建右心室流出道。　　结果 :无手术和住院死亡。平均随访 2 3± 13个月。超声心动图检查肺动脉瓣功能良好 ,无明显反流。右心室至肺动脉平均流速及压差均较术前明显下降 ,P均 <0 .0 1。　　结论 :TOF/ APV的外科矫治应强调对扩张肺动脉的处理 ,彻底解除气道压迫。应用同种肺动脉重建右心室流出道方法简单 ,效果良好。     

Absent Pulmonary Valve: Echocardiographic Features

Congenital absence of the pulmonary valve, a rare anomaly, is characterized by absent or rudimentary pulmonary valve, often with annular stenosis, and aneurysmal dilatation of the pulmonary arteries. This defect is most commonly an accompaniment of tetralogy of Fallot but occasionally occurs alone. Four patients with this abnormality were examined by two-dimensional echocardiography at the Mayo Clinic. Doppler echocardiography provided hemodynamic assessment of the magnitude of outflow obstruction and valve regurgitation. The two-dimensional echocardio-graphic and Doppler features of absent or rudimentary pulmonary valve provided diagnostic information sufficient to proceed directly to surgical correction.     

Prenatal Diagnosis and Hemodynamics in Tetralogy of Fallot, Absent Pulmonary Valve with Endocardial Fibroelastosis, and Hydrops Fetalis

Tetralogy of fallot with absent pulmonary valve is rare. We report the antenatal diagnosis of this condition in early mid-semester. In this unusual case, the patient presented with hydrops fetalis. The pathophysiology is discussed. (ECHOCARDIOGRAPHY, Volume 10, November 1993)     

Late Postoperative Prosthetic Pulmonary Valve Endocarditis in a 13-Year-Old Girl with Repaired Tetralogy of Fallot

Prosthetic pulmonary valve endocarditis has infrequently been described outside large cohort reviews, which have typically focused on infections of the left-sided heart valves. Hence, the pathogenesis, clinical presentation, and management principles of prosthetic pulmonary valve endocarditis have not been well differentiated from those of infected aortic and mitral valves. More patients with repaired tetralogy of Fallot are reaching adulthood and will need pulmonary valve implantation. Consequently, a focus on this infrequent but serious cardiac infection is needed, to learn what characteristics might distinguish it from infections of left-sided heart valves.We report the case of a 13-year-old girl with repaired tetralogy of Fallot who presented with fever and nonspecific symptoms. The patient initially failed to meet the Duke criteria for endocarditis but was then found to have endocarditis of her prosthetic pulmonary valve. We explanted the valve and replaced it with a pulmonary homograft, after which the patient had no infectious sequelae. In addition to presenting the patient''s case, we review the literature on surgically inserted prosthetic pulmonary valves and discuss the primary management concerns when those valves become infected with endocarditis.     

Fresh Autologous Pericardium to Reconstruct the Pulmonary Valve at the Annulus When Tetralogy of Fallot Requires a Transannular Patch at Midterm

Tetralogy of Fallot often requires reconstruction of the right ventricular outflow tract with a transannular patch (TAP), but this renders the pulmonary valve incompetent and eventually leads to right ventricular dysfunction.We retrospectively evaluated the efficacy of a reconstructed pulmonary valve and annulus in 70 patients who underwent, from December 2006 through December 2010, complete correction of tetralogy of Fallot. We divided the 70 patients into 2 groups in accordance with whether they required (n=50) or did not require (n=20) a TAP. We used autologous untreated pericardium to fashion the TAP and to create both an annulus of the correct size and a competent pulmonary valve with native leaflets. We evaluated the efficiency of this procedure both functionally and anatomically.The median age of the patients was 11 years (range, 2–38 yr). There were 56 males, with no significant difference in sexual distribution between groups. The clinical follow-up was 88% for 57.5 months, and the echocardiographic follow-up was 80% for 36 months. There was no significant difference in outflow gradient or in the occurrence of pulmonary insufficiency between the TAP group (none, 31; mild, 12; moderate, 6; and severe, 1) and the No-TAP group (none, 16; moderate, 2; and severe, 2) (P=0.59). Nor was there any thickening or calcification in the constructed valves.We conclude that pulmonary valves constructed of untreated autologous pericardium performed as well as native valves after total tetralogy of Fallot correction at midterm.     

同种主动脉跨环补片用于法鲁四联症根治术

本文报告６例法鲁四联症（ＴＯＦ）采用同种主动脉瓣（ＨＡＶ）保留二尖瓣前叶法行根治术，ＨＡＶ采用液氮保存，全组均为男性，年龄２．５－８岁，均有肺动脉发育不全。跨环补片采用ＨＡＶ主动脉壁加宽肺动脉，二尖瓣前叶加宽右室流出道；６例全部存活，无并发症；术后超声心动图（ＵＣＧ）示右室流出道疏通满意，无肺动脉瓣返流。     

Percutaneous Pulmonary Valve Placement

Patients with congenital heart disease and pulmonary valve disease need multiple procedures over their lifetimes to replace their pulmonary valves. Chronic pulmonary stenosis, regurgitation, or both have untoward effects on ventricular function and on the clinical status of these patients. To date, all right ventricle–pulmonary artery conduits have had relatively short lifespans. Percutaneous pulmonary valve implantation, although relatively new, will probably reduce the number of operative procedures that these patients will have to undergo over a lifetime. Refinement and further development of this procedure holds promise for the extension of this technology to other patient populations.     

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient''s anginal symptoms were relieved after pulmonary arterioplasty.Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension—a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.     

N-Butyl Cyanoacrylate Embolization of a Peripheral Pulmonary Artery Aneurysm in a Patient with Patent Ductus Arteriosus

N-Butyl cyanoacrylate embolization of peripheral pulmonary artery aneurysms has rarely been performed. This report presents a young woman with a history of patent ductus arteriosus and massive hemoptysis secondary to pulmonary artery aneurysm, successfully managed by embolotherapy using n-Butyl cyanoacrylate. This is the first report of n-Butyl cyanoacrylate embolization treatment of a bleeding pulmonary artery aneurysm in the setting of patent ductus arteriosus. The pertinent literature has been reviewed and the clinical background and the procedure have been explained.     

法洛四联症外科根治手术治疗6个月以内患儿54例经验总结

目的总结54例6个月以内婴幼儿行法洛四联症(tetrology of Fallot,TOF)外科根治手术的经验。方法 2006年1月至2012年12月广东省人民医院对54例<6个月的TOF患儿进行一期根治手术,手术时月龄为(4.94±0.40)个月,体质量为(5.98±0.30)kg。其中男34例,占63%;女20例,占37%。无症状患儿4例,占7.4%。有症状患儿50例,占92.3%;其中,缺氧发作的16例,占30%。术前均经过超声心动图和心脏螺旋计算机断层扫描检查确诊,M率为1.76(0.94～3.09)。手术均在中低温体外循环下进行,94.4%患儿行跨瓣环补片扩大右心室流出道,经右心室或右心房切口修补室间隔缺损,同期完成其他合并畸形的矫治。结果总住院病死率为5.6%(3/54),主动脉阻断时间为(58.3±12.2)min,体外循环时间为(87.2±11.6)min。肺动脉瓣轻度反流38例,占70.4%(38/54)。无Ⅲ度房室传导阻滞,轻度右心室流出道梗阻5例。结论 TOF矫治年龄可以前移至在6个月以内进行,并可以获得良好的手术效果和较低的手术病死率。     

Outcomes of Percutaneous Closure of Patent Ductus Arteriosus Accompanied With Unilateral Absence of Pulmonary Artery

Background

Limited data have reported the outcomes of percutaneous closure of patent ductus arteriosus (PDA) in patients with unilateral absence of pulmonary artery (UAPA). This study aimed to evaluate the symptomatology, diagnosis and therapy, especially the transcatheter closure of PDA in patients with PDA associated with UAPA.

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion.We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.     

Abnormal Agitated Saline Study Results after Tetralogy of Fallot Repair

Residual intracardiac or extracardiac shunting is frequently seen in patients with repaired congenital heart disease and can cause systemic hypoxemia. We present the case of an adult with tetralogy of Fallot who underwent two corrective surgical procedures as a child with subsequent pulmonary valve replacement due to residual pulmonary insufficiency. Further details of her operative history were otherwise unknown. After being lost to follow‐up for many years, she presented with unexplained cyanosis and a markedly abnormal agitated saline study on transthoracic echocardiography in which only the left heart filled after contrast administration. We review the differential diagnosis for such a presentation and discuss the utility of cardiac magnetic resonance imaging (MRI) to define the etiology. Cardiac MRI, a frequently used imaging modality to longitudinally assess adult patients with congenital heart disease, provides excellent image quality of cardiac structures and the ability to perform angiography in a variety of imaging planes. These advantages render cardiac MRI, a useful modality to determine the etiology of unexplained cyanosis in these patients as both intracardiac and extracardiac shunts can be detected.     

Chronotropic Response and Pulmonary Function are Associated with Exercise Performance in Children and Adolescents with Repaired Tetralogy of Fallot Independent of Cardiac Function

Objective: The determinants of exercise capacity in repaired tetralogy of Fallot (rTOF) are multifactorial and remain incompletely understood. This study sought to evaluate the association of chronotropic response with exercise parameters and investigate the determinants of heart rate reserve (HRR) in a cohort of children and adolescents with rTOF. Design: We retrospectively analyzed patients with rTOF, age 8–18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) for research purposes. Linear regression models were performed to test associations among clinical, CMR and CPET parameters. Outcomes included percent-predicted maximum VO₂ (%mVO₂) and HRR. Results: A total of 148 patients were included (mean age 12.3 ± 3.1 years). The majority of patients had TOF with pulmonary stenosis (80%) and underwent transannular patch TOF repair (78%). Median age at surgical repair was 4.2 months (IQR 1.2, 8.4). There was preserved RV ejection fraction (60.4 ± 8.3%) and moderate pulmonary insufficiency (regurgitant fraction 35.2 ± 16.6 %). On CPET, %mVO₂ was overall diminished (76.5 ± 17.9 %), and % predicted forced vital capacity (FVC) was diminished on spirometry. HRR, FVC and ability to reach maximum effort were independently associated with greater %mVO₂. FVC, net forward flow in the main pulmonary artery/m² , and reaching maximum effort were associated with greater HRR, independently of RV volume, degree of PI and RV ejection fraction. Conclusions: In patients with rTOF, HRR and pulmonary function (FVC) are more important contributors to exercise performance than right ventricular function. Interventions to improve chronotropic health and pulmonary function should be explored.