超声心动图对左心发育不良综合征术前诊断及术后评价的意义

目的 总结1998年在美国贝勒医学院工作期间,左心发育不良综合征（HLHS）患者改良NorwoodⅢ期心脏重建手术前后超声心动图诊断的资料和经验,评价其价值并为国内同行借鉴。方法 HLHS患者18例,Norwood I期手术10例,Ⅱ期手术6例,Ⅲ期手术2例。术前超声心动图结果与术中外科诊断比较,分诊断符合、诊断不符和轻微差别。结果 NorwoodI期手术10例患者均无术前心导管检查,超声心动图术前诊断与术中外科诊断达到100％的诊断符合。Ⅱ期手术患者6例,超声心动图对I期手术术后评价的诊断符合率为100％,分支肺动脉的评价33．3％诊断符合,66．7％有轻微差别。Ⅲ期手术的2例病患者超声心动图与术中诊断均相符。结论 单靠超声心动图能够确立HLHS的诊断并为NorwoodI期手术提供足够的信息,对NorwoodⅡ期和Ⅲ期术前及术后诊断超声心动图能够提供大量正确的信息,但对分支肺动脉及肺静脉的精确解剖及压力数据的测量存在局限性。     

Hybrid Transcatheter–Surgical Palliation

The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional–surgical “hybrid approach” as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex. Between 1998 and April, 2006, 58 newborns underwent ductal stenting and bilateral PAB. These patients underwent surgical bilateral PAB initially, followed by percutaneous duct stenting; the only exception to this were patients in whom duct stenting was performed as a rescue procedure. Various balloon-expandable and self-expandable stents with different widths and lengths were used during the 8-year period of this study. Balloon dilatation of the atrial septum was performed when indicated. This included 5 patients in whom the atrial septum was stented. Aortic arch reconstruction (AAR) combined with a bidirectional cavopulmonary connection (BCPC) was performed at a median age of 4.8 months (range, 2.6–7.5), and total cavopulmonary connection (TCPC) was performed at a median age of 3.1 years (range, 2.5–4). Nine patients were listed for heart transplantation (HTX) and transplanted with AAR when a donor heart was available. Depending on growth of left ventricular structures, biventricular repair (BVR) was performed at a median age of 7.1 months (range, 3.5–10). Overall, 8 of 58 patients (13.8%) treated by the transcatheter–surgical hybrid approach died during the study period. The mortality rate for duct stenting was 1.7% (l/58), and it was 1.7% for bilateral PAB as well. Twenty-seven patients received an AAR/BCPC; 2 of them died (7.4%). Additionally, 1 of 2 patients with AAR/BCPC died while on the waiting list for HTX, resulting in a total mortality rate of 11% with an actuarial survival rate of 89%. One patient is still awaiting AAR + BCPC. Three patients died while on the waiting list for HTX despite successful bilateral PAB and duct stenting. The 30-day mortality rate for TCPC (n = 11), HTX (n = 8), and 18 patients with BVR was 0. The actuarial survival rate for patients with BVR is 93%. Postnatal transcatheter–surgical hybrid palliation expands the surgical options for newborns with HLH. Using hybrid palliation, Norwood stage I operation can be avoided in the neonatal period, the waiting period for children scheduled for cardiac transplantation can be extended, and observation for left ventricular growth suitable for biventricular repair as well.     

A Risk Assessment Scoring System Predicts Survival Following the Norwood Procedure

No one set of characteristics has been consistently predictive of perioperative mortality and morbidity associated with the Norwood procedure. The purpose of the current study is to further validate a scoring system shown to be predictive of mortality following the Norwood procedure. We performed a retrospective review of all infants with the diagnosis of hypoplastic left heart syndrome (HLHS) who underwent the Norwood procedure at St. Louis Children’s Hospital from July 1, 1994, to December 31, 2002. A weighted score for each of six factors comprised the scoring system. The factors included ventricular function, tricuspid regurgitation, ascending aortic diameter, atrial septal defect blood flow characteristics, blood type, and age. A score of ≥7 points indicated lower reconstructive mortality risk, and a total score of <7 points indicated a higher mortality risk. A total of 57 patients were analyzed. Twenty-five infants (44%) had a low risk score. These infants had a significantly greater survival at 48 hours compared to infants with a score of <7 (92 vs 75%, p < 0.05). Infants with a high risk score had a significantly greater relative risk of mortality at 48 hours [OR = 2.04; confidence interval (CI) 1.04–4.00; p = 0.036]. The area under the receiver operating characteristic (ROC) curve is 0.8534 (95% CI, 0.78–0.922). This suggests that the scoring system has a very good degree of discriminatory power in selecting children who did not survive. Based on the results of the ROC, a cutoff score of >7 gives the best sensitivity and specificity for survival. When applied retrospectively, the survival outcomes predicted by our scoring system significantly correlated with actual outcomes. This supports the conclusion that a specific population of HLHS patients may have a higher mortality risk independent of surgical technique and postoperative care based on factors that can be assessed preoperatively.     

Thrombus Formation in the Native Aortic Root in Patients with Hypoplastic Left Heart Syndrome

Thrombus formation in the native aortic root is a rare but potentially life-threatening complication in patients with hypoplastic left heart syndrome. The native aortic root in these patients serves as a conduit for the retrograde filling of the coronary arteries. Thrombus in this location could result in myocardial ischemia. We present three cases of native aortic root thrombosis at our institution with clinical presentations ranging from asymptomatic to sudden cardiac arrest. Our experience with these patients argues for careful and consistent evaluation of the native aortic root regardless of clinical symptoms.     

Resuscitation and Perioperative Management of the High‐risk Single Ventricle Patient: First‐stage Palliation

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high‐risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first‐stage clinical management strategies are reviewed.     

Creation of high position fetal balloon atrial septoplasty for hypoplastic left heart syndrome and highly restrictive atrial septum: A case report and literature review

BackgroundFetal balloon atrial septoplasty (BAS) is performed through the restrictive foramen ovale in fetal cases with established hypoplastic left heart syndrome (HLHS) and an intact or highly restrictive atrial septum (RAS).MethodsIn the current report, we present a case of high position BAS in a fetus with HLHS/RAS.ResultsEchocardiography confirmed an adequate atrial opening above the foramen ovale and fetal pleural effusion resolved spontaneously 1 day after the procedure.ConclusionTo the best of our knowledge, the creation of a high position hole in the thinner part of the atrial septum, instead of the restrictive tiny hole, has not been reported in fetal cases with HLHS/RAS.     

A Different Therapeutic Strategy for Severe Tetralogy of Fallot with Origin of the Left Pulmonary Artery from the Ascending Aorta: Stenting of the Right Ventricular Outflow Tract before Complete Repair

The origin of pulmonary artery branches (particularly the left pulmonary artery) from the ascending aorta is a rare condition. We detected prominent hypoplasia of the main and right pulmonary arteries in a 3.5‐month‐old 3.7 kg female infant who had tetralogy of Fallot with origin of the left pulmonary artery in the ascending aorta. In order to ensure the development of the right pulmonary artery, a stent was put in that extended from the right ventricular outflow tract to the right pulmonary artery. During follow‐up, after the patient's right pulmonary artery had developed sufficiently, a complete repair surgery was done. It is common practice for patients with abnormal origin of the left pulmonary artery to perform the complete repair using the direct reimplantation technique. However, we think that another possibility is to implant the stent in patients with hypoplastic pulmonary artery and branches in the early stages, wait for a short period of time and perform the complete repair surgery before permanent pulmonary hypertension develops.     

Norwood术后心导管造影检查的应用及介入治疗

目的 探讨Norwood术后心导管造影检查结果以及介入治疗方法和疗效.方法 13例病婴均为Norwood术后而尚未行Glenn手术者,其中12例接受了介入治疗.结果 13例中6例出现新主动脉弓再狭窄,行主动脉成形术.1例左肺动脉狭窄,行左肺动脉球囊扩张术,3个月后左肺动脉置入支架.2例Sano-Shunt狭窄和1例BT-Shunt狭窄,均置入支架治疗,改善肺血流.4例合并体肺动脉侧支,其中3例采用Coil行侧支血管堵塞术,另1例于次日Glenn手术术中结扎.结论 Norwood术后血流动力学异常的发生率很高,术后造影和血流动力学的评估以及对新主动脉弓或肺动脉狭窄的介入治疗是必要的.

Abstract：

Objective To evaluate the outcome of the cardiac catheter angiograplasty for patients after Norwood procedure. Methods 13 patients, who had undergone Norwood procedure (prior to Glenn procedure)received cardiac catheterization and angiography. lnterventional therapy was performed in 12 patients as indicated. Results Angioplasty was carried out for re-coarctation in 6 of the13 patients. 1 patient with left pulmonary artery stenosis received balloon dilation and 3 months later a stent implantation. A stent was implanted to improve the pulmonary blood flow in 2 patients with Sano-Shunt stenosis and 1 patient with BT-Shunt stenosis. Transcatheter coil embolization was performed in 3 of the 4 patients with systemic to pulmonary collateral vessels. The other one was scheduled for Glenn procedure the next day. Conclusion The incidence of hemodynamic restrictions after Norwood procedure is high (12 of 13 patients). Postoperative angiography and hemodynamic assessment for diagnosis and interventional treatment for new pulmonary artery or aortic arch stenosis is necessary after Norwood procedure.