Heartmate II Implantation with Right Coronary Bypass Grafting in Ischemic Cardiomyopathy with "Fixed" Pulmonary Hypertension: Treatment Strategy to Protect Right Ventricular Function

Abstract   A 49-year-old male patient suffering from end-stage ischemic cardiomyopathy with a left ventricular ejection fraction below 15% was presented to redo coronary artery bypass grafting (CABG). Coronary angiogram demonstrated an occluded left anterior descending artery and occluded right coronary artery, perfused retrogradely from the circumflex artery. Since positron emission tomography did not demonstrate viable left ventricular myocardium except for the basis of the left ventricle, CABG was considered futile. Cardiac transplantation was contra-indicated due to pharmacologically unresponsive pulmonary artery hypertension. The patient successfully underwent left ventricular assist device implantation in combination with right coronary artery revascularization. The article reflects the regimen of right ventricular preservation in this patient. (J Card Surg 2010;25:116-119)     

牛颈静脉带瓣管道重建犬右心室流出道的血流动力学研究

目的观察自制牛颈静脉带瓣管道重建犬右心室流出道后的血流动力学性能. 方法应用经戊二醛处理的牛颈静脉带瓣管道对7只犬行肺动脉与右心室连接,重建右心室流出道前后测定肺血流动力学,行超声心动图检查牛颈静脉带瓣管道通畅情况. 结果重建术后1年7只犬均存活.重建右心室流出道前后肺动脉收缩压、舒张压和平均压均无明显改变,右心室舒张压无明显变化,收缩压和平均压显著增加(P<0.01).术后超声心动图检查瓣膜关闭良好,无明显反流,跨瓣压差小;术后1年超声心动图检测发现全部带瓣管道通畅,未见明显血栓形成,除1只犬瓣叶活动稍差合并轻度反流外,其余犬瓣叶活动良好.心导管测压显示肺动脉与右心室之间压差为3～19 mm Hg(1kPa=7.5mmHg),管道内舒张压显著高于右心室舒张压,右心室造影显示牛颈静脉带瓣管道和肺动脉无明显梗阻.结论戊二醛处理牛颈静脉带瓣管道重建右心室流出道具有良好的血流动力学性能.     

Pediatric lung transplantation for pulmonary hypertension and congenital heart disease.

Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.     

Molecular and functional mechanisms of right ventricular adaptation in chronic pulmonary hypertension.

BACKGROUND: Chronic pulmonary hypertension can lead to compensatory changes in the right ventricle. In this study, the adaptive mechanisms of the right ventricle in the setting of pulmonary hypertension were assessed at the molecular and functional level using a canine model of monocrotaline pyrrole-induced pulmonary hypertension. METHODS: Animals underwent pulmonary artery catheterization to measure pulmonary hemodynamics before and 8 weeks after an injection of monocrotaline pyrrole, 3 mg/kg (n = 8) or placebo (n = 8) (controls). Systolic function was assessed with load-insensitive means (preload-recruitable stroke work). Myocardial biopsy specimens were collected to analyze membrane alpha1- and beta-adrenergic receptor density and adenylate cyclase activity. RESULTS: Eight weeks after injection, significant increases in pulmonary hemodynamic indices were noted in monocrotaline-injected dogs. Significant increases in right ventricular preload-recruitable stroke work were also observed in these animals compared with controls and occurred in association with significant increases in right ventricular alpha1- and beta-adrenergic receptor density and isoproterenol hydrochloride-stimulated adenylate cyclase activity. No significant differences in basal adenylate cyclase activity in the right ventricle were noted between the two groups. CONCLUSIONS: These data suggest that alterations in right ventricular function in the setting of chronic pulmonary hypertension may partially be due to changes in myocardial adrenergic receptor signaling.     

Hemodynamic effect of a low-resistance artificial lung in series with the native lungs of sheep

BACKGROUND: An artificial lung with 1 to 6 month work life could act as a bridge to transplantation. A pumpless artificial lung has been developed. METHODS: The artificial lung was placed in series with the native lungs of adult sheep. Hemodynamics were observed, as the right ventricle generated flow through the device. Through a left thoracotomy, two 20-mm grafts were anastomosed in an end-to-side fashion to the pulmonary artery. The grafts were externalized, and directed flow through the chest wall, to the extracorporeal lung. The animals were recovered, weaned from the ventilator, and when standing, flow was diverted through the device. RESULTS: Five of 7 animals survived 24 hours with 75% to 100% of the cardiac output diverted through the device. All animals were active, with interest in food and water, and able to stand. CONCLUSIONS: The right ventricle perfused the artificial lung with 75% to 100% of the cardiac output for 24 hours. This device demonstrates the feasibility of a pumpless pulmonary assist device relying on the right ventricle for perfusion.     

Management of pulmonary hypertension in the operating room

Pulmonary artery hypertension is defined as persistent elevation of mean pulmonary artery pressure > 25 mm Hg with pulmonary capillary wedge pressure < 15 mm Hg or elevation of exercise mean pulmonary artery pressure > 35 mm Hg. Although mild pulmonary hypertension rarely impacts anesthetic management, severe pulmonary hypertension and exacerbation of moderate hypertension can lead to acute right ventricular failure and cardiogenic shock. Knowledge of anesthetic drug effects on the pulmonary circulation is essential for anesthesiologists. Intraoperative management should include prevention of exacerbating factors such as hypoxemia, hypercarbia, acidosis, hypothermia, hypervolemia, and increased intrathoracic pressure; monitoring and optimizing right ventricular function; and treatment with selective pulmonary vasodilators. Recent advances in pharmacology provide anesthesiologists with a wide variety of options for selective pulmonary vasodilatation. Pulmonary hypertension is a major determinant of perioperative morbidity and mortality in special situations such as heart and lung transplantation, pneumonectomy, and ventricular assist device placement.     

Assessment of cardiac performance using Tei indices in patients undergoing pulmonary thromboendarterectomy

BACKGROUND: This study was designed to evaluate left and right ventricular performance using Tei indices in patients with severe chronic thromboembolic pulmonary hypertension undergoing pulmonary thromboendarterectomy (PTE). The Doppler-derived indices are easily measurable indicators of ventricular function based on nongeometric assessment, which helps overcome some of the difficulties entailed in the geometric assessment of left ventricular (LV) and right ventricular (RV) function in pulmonary hypertension. METHODS: The indices were derived for 24 patients (aged 54+/-14 years) before and after PTE. Calculation of these indices was based on the duration of two time intervals using the formula (A - B)/B, where A is the interval between cessation and onset of mitral inflow (or tricuspid inflow) and B is LV or RV ejection time. In addition, LV and RV end-diastolic and end-systolic chamber areas were determined using two-dimensional echocardiography, and systolic function was calculated. Mean pulmonary artery pressure was determined invasively. RESULTS: PTE led to a significant reduction of mean pulmonary artery pressure (46+/-10 versus 25+/-6 mm Hg; p < 0.05). LV and RV indices were abnormally high before surgery, declined significantly afterwards, and then almost matched normal values (0.61+/-0.26 versus 0.37+/-0.18; p < 0.05 and 0.55+/-0.22 versus 0.37+/-0.13; p < 0.05). Geometric assessment of the left and right ventricle also showed impaired systolic function before PTE, with significant improvement after surgery. CONCLUSIONS: LV and RV Tei indices allow a quantitative assessment of ventricular function in patients undergoing PTE. Lower indices after surgery reflect an improvement of the previously impaired cardiac function. Our results emphasize the value of PTE in the treatment of chronic thromboembolic pulmonary hypertension.     

肺移植治疗儿童肺动脉高压的临床疗效

目的 探讨同种异体肺移植治疗儿童肺动脉高压(PAH)的临床疗效.方法 受者例1、2为特发性肺动脉高压( IPAH)患儿,均经右心导管术确诊为IPAH,术前心功能不全Ⅳ级,肺动脉收缩压、平均压分别为110、70 mm Hg(1 mm Hg =0.133 kPa)和148、72 mm Hg,在全麻体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,术中ECMO支持时间分别为550 min和450 min,出血量分别为3000 ml和1200 ml.受者例3为先天性心脏病室间隔缺损合并艾森曼格综合征心内直视探查术后,超声心动图(UCG)提示先天性心脏病室间隔缺损,双向分流,肺动脉收缩压、平均压为110、60 mm Hg,在全麻低温体外循环(CPB)下行室间隔缺损修补术同期右侧单肺移植术,术中CPB时间244 min.3例受者与供者体型较匹配,ABO血型相同.结果 受者例1、2术后ECMO分别维持16h、13 h后撤离,术后第3、4天均出现不同程度的血流动力学不稳定,诊断为急性左心衰,均于术后第3、6天行气管切开呼吸机辅助正压通气,经强心、利尿、扩血管等治疗,分别于术后第33天、12天脱离呼吸机.受者例3术后3天内移植肺出现中等程度再植反应性肺水肿,术后第7天气管切开,第12天撤离呼吸机;术后第14天出现急性排斥1次,治疗后缓解.3例受者术后UCG提示心脏形态和心功能明显改善,受者例3室间隔缺损修补完整,无残余分流.分别于术后第93天、32天、62天康复出院,心功能均达Ⅰ级,肺动脉收缩压、平均压分别降为54、32 mm Hg,60、36 mm Hg和53、39 mm Hg.术后已随访41、21、82个月,患儿正常工作学习,至今生活质量良好.结论 对终末期经内科保守治疗效果欠佳的PAH患儿行肺移植能很好改善生活质量.     

Pediatric heart transplantation after operations involving the pulmonary arteries

A prohibitive perioperative mortality has been previously ascribed to pediatric heart transplantation after palliative operations for congenital heart disease involving the pulmonary arteries. Of 46 children who have undergone heart transplantation at our institution between June 1984 and February 1990, 7 (15%; mean age 8 +/- 3 years; range 1 to 18 years) have previously undergone such operations: right ventricle to pulmonary artery conduit/homograft for levo-transposition of the great arteries (2), Waterston shunt for tricuspid and pulmonary atresia (1), pulmonary artery banding for single ventricle (1), Fontan procedure for single ventricle (1), first-stage Norwood procedure for hypoplastic left heart syndrome (1), and classic right Blalock-Taussig shunt for atrioventricular canal with pulmonic stenosis (1). Three categories of pulmonary artery anatomy that require different approaches to reconstruction at the time of transplantation are recognized: abnormalities of position, pulmonary outflow obstruction, and previous systemic- or atrial-pulmonary connections. At operation, individualized pulmonary arterial reconstruction was employed, including use of previously created right ventricular-pulmonary artery conduits/homografts and angioplasty (with and without pericardial patches). Transplantation was successful in all patients. Posttransplant right ventricular-pulmonary artery pressure gradients and pulmonary vascular resistance indices were acceptable, with a tendency to decrease with time. Two patients had critical right ventricular failure postoperatively; one of them required support with extracorporeal membrane oxygenation. There was no perioperative mortality, with three deaths occurring from 5 to 39 months after transplantation. All surviving patients are in New York Heart Association functional class I. Techniques borrowed from the repair of congenital cardiac lesions can be applied to subgroups of children undergoing heart transplantation. Additional length of donor aorta and pulmonary artery should be harvested for possible use in designing pulmonary artery connections. Previous palliative operations involving the pulmonary arteries with associated complex pulmonary artery anatomy are not of themselves an insurmountable obstacle to successful heart transplantation.     

Left ventricle is better suited as pulmonary ventricle in simple transposition with severe pulmonary hypertension

BACKGROUND: The conventional treatment of transposition of great arteries with prepared left ventricle is an arterial switch operation. This, in our experience, does not hold for patients with transposition of great arteries with intact ventricular septum where the left ventricle continues to be prepared secondary to severe pulmonary arterial hypertension without an immediately reversible cause. METHODS: Ten infants with D-transposition of the great arteries with essentially intact interventricular septum and severe pulmonary arterial hypertension underwent surgical treatment. Age ranged from 3 to 6 months (mean, 4.2 months). One of these patients had a large ductus with left to right shunting but the others had no intra- or extracardiac shunt to account for their pulmonary hypertension. All 10 had "prepared" left ventricles. The first 4 children underwent an arterial switch operation. Uneventful surgery was followed by prolonged ventilator dependence in all 4 with occurrence of severe pulmonary arterial hypertension every time weaning from ventilator was attempted. This was accompanied by metabolic acidosis and features of right heart failure. Only 1 patient with large ductus could be extubated and discharged from hospital. Subsequently, the other 6 infants underwent a Senning repair. RESULTS: There was no early mortality. All patients were separated from mechanical ventilation within 48 hours of surgery without blood gas derangement or heart failure despite elevated pulmonary artery pressure in all. The child with the arterial switch operation has pulmonary artery pressure of 50% systemic 4 years following repair; although among the Senning group, 2 patients continue to have pulmonary artery pressure more than 60% of systemic and 4 have normal pulmonary artery pressure at a mean follow-up of 1 year. CONCLUSIONS: Atrial level repairs seem to perform better than arterial level repairs in children having TGA with persistent pulmonary artery hypertension without a correctable cause. Better tolerance of pulmonary arterial hypertension in this group is probably consequent to the superior ability of the left ventricle to tolerate a pressure load in the early postoperative period.     

Continuous pulmonary perfusion during cardiopulmonary bypass prevents lung injury in infants

BACKGROUND: Lung injury after cardiopulmonary bypass is a serious complication for infants with congenital heart disease and pulmonary hypertension. Excessive neutrophil sequestration in the lung occurring after reestablishment of pulmonary circulation implies that interaction between neutrophils and pulmonary endothelium is the major cause of lung injury. METHODS: Thirty infants with either ventricular septal defect or atrioventricular septal defect and with pulmonary hypertension were enrolled in this study. We performed continuous pulmonary perfusion during total cardiopulmonary bypass on 16 patients (perfused group) and conventional cardiopulmonary bypass on 14 patients (control group). PaO2/FiO2 and neutrophil counts were assessed from immediately before surgery to 24 hours after termination of cardiopulmonary bypass. RESULTS: PaO2/FiO2 was higher in the perfused group than in the control group, and the difference was significant throughout the study period. Neutrophil counts decreased below prebypass values in both groups at 30 minutes after aortic unclamping, and the difference was significant in the control group but was not in the perfused group. Duration of postoperative ventilatory support was significantly less in the perfused group. CONCLUSIONS: Our study demonstrates that arrested pulmonary circulation during cardiopulmonary bypass is the major risk factor of lung injury and that continuous pulmonary perfusion is effective in preventing lung injury.     

Single lung transplantation in rats with chemically induced pulmonary hypertension.

Physiologic effects of single lung transplantation on pulmonary hypertension were studied in rats with monocrotaline-induced pulmonary hypertension. Inbred rats treated with monocrotaline (40 mg/kg) received a left lung isograft from a normal donor 2 weeks later, when pulmonary hypertension became significant (transplant group; n = 6). These rats and control rats treated with monocrotaline (mediated control group; n = 11) or vehicle alone (normal control group; n = 9) were followed up weekly by metabolic treadmill testing for exercise tolerance and oxygen consumption up to 6 weeks after monocrotaline (4 weeks after transplantation), when all rats underwent hemodynamic and histologic examinations. Whereas maximal oxygen consumption and exercise tolerance consistently deteriorated in the medicated control group of rats, indices in the transplant group stopped deteriorating 2 weeks after lung transplantation and remained at levels similar to those of normal control rats. Severe pulmonary hypertension (68 +/- 19 mm Hg) and right ventricular hypertrophy (right ventricular/left ventricular weight ratio, 0.95 +/- 0.19) were confirmed in medicated control rats in contrast to transplant animals, in which these two indices remained at normal control levels. Whereas left-to-right lung perfusion ratio was constant among rats not receiving transplants (0.69 +/- 0.16), it was significantly elevated (2.27 +/- 0.65; p less than 0.001) in those receiving transplants, suggesting preferential flow through the lung isograft. The results suggest that, in the early phase of pulmonary hypertension, single lung transplantation shifts pulmonary perfusion to the grafted lung, avoiding right ventricular pressure overload and thereby preserving exercise tolerance at a nearly normal level in rats with monocrotaline-induced pulmonary hypertension.     

肺移植受者围手术期肺动脉高压的调控（附视频）

肺移植受者多为终末期肺病患者。无论是肺实质还是肺血管病变,都有不同程度肺动脉高压。肺动脉高压可能导致右侧心力衰竭,严重影响肺移植预后。因此,必须充分了解肺移植围手术期影响肺血管阻力的因素,包括麻醉诱导、机械通气、单肺通气、阻断一侧肺动脉以及手术操作等。可通过应用静脉或吸入的肺血管扩张药降低肺血管阻力以及正性肌力药支持右心功能等进行调控。     

Right ventricular dynamic cardiomyoplasty for the univentricular heart with pulmonary hypertension

OBJECTIVES: We conducted an acute experimental study to test the feasibility of dynamic cardiomyoplasty in a setting of modified Fontan procedure for univentricular heart with pulmonary hypertension to obtain a possible proxy for high-risk Fontan candidates. METHODS: After electrical preconditioning of the left latissimuss dorsi for 6 weeks in 8 dogs, the right ventricular cavity was totally obliterated with concomittent closure of the tricuspid valve and right pulmonary artery. Modified Fontan circulation was established with the aortic homograft anastomosed between the right atrium and pulmonary trunk, incorporated with a pericardial pouch as a compression chamber (neoright ventricle) fixed onto the epicardial surface of the ventricle. After cardiopulmonary bypass termination, a latissimus dorsi was applied to wrap the pericardial pouch and ventricle clockwise and stimulated with a trained-pulse (25 Hz) at 1:1 synchronization ratio with cardiac beats. RESULTS: Profound right heart failure was noted during Fontan circulation in increased pulmonary vascular resistance (11 +/- 3.2 Wood units), whereas graft pacing showed significant augmentation of systolic pulmonary pressure by 54 +/- 12%, the mean pulmonary flow by 68 +/- 23%, and aortic pressure by 23 +/- 5% at a physiological range of central venous pressure (13.2 +/- 0.7 mmHg). Right heart function curve analysis confirmed marked augmentation of right heart performance, restoring almost normal pulmonary circulation. These functional benefits were sustained up to 4 hours in 4 animals until experiments were terminated. CONCLUSIONS: Dynamic cardiomyoplasty in a modified Fontan procedure is a viable surgical option for univentricular heart, not a Fontan candidate.     

Hemodynamic unloading leads to regression of pulmonary vascular disease in rats

OBJECTIVE: Treatment options for patients with advanced pulmonary vascular disease caused by a congenital heart defect are still mainly limited to heart-lung transplantation or lung transplantation with repair of the cardiac lesion. Because we have previously shown that the structural changes associated with pulmonary hypertension can be reversed by stress unloading in an organ culture model, we now investigate whether hemodynamic unloading will lead to regression of pulmonary vascular disease in the intact animal. METHODS: Right middle and lower lobectomy and monocrotaline injection were performed in Lewis rats (n = 22) to cause pulmonary vascular disease from a combined hemodynamic and toxic injury. Twenty-eight days later the left lungs were examined (n = 10) or exposed to normal pulmonary artery pressure for an additional 14 (n = 5) or 28 (n = 7) days by transplantation into healthy recipients. Pulmonary artery pressure, ventricular weight, and pulmonary artery morphology were evaluated in each group. RESULTS: Pulmonary hypertension (50 vs 16 mm Hg; P <.001) and right ventricular hypertrophy (right ventricular/left ventricular weight 0.69 vs 0.32; P <.001) associated with pulmonary artery medial hypertrophy (28.2% vs 7.2% wall thickness; P <.001) and muscularization of small pulmonary arteries (92.3% vs 19.4%; P <.001) developed by day 28 (compared with untreated controls). However, transplantation into healthy recipients effectively unloaded the lungs (mean pulmonary artery pressure 17 and 24 mm Hg at 14 and 28 days after transplantation) and resulted in progressive normalization of medial hypertrophy (15.6% and 12.1% at 14 and 28 days) and muscularization (65.1% and 42.2% at 14 and 28 days) relative to nontransplanted controls (P <.005 in each case). CONCLUSIONS: Hemodynamic unloading of lungs with pulmonary vascular disease results in progressive normalization of pulmonary artery structure. These results are the first to provide a rationale for attempting to induce regression of pulmonary vascular disease by pressure unloading of the pulmonary circulation. Methods to mechanically unload the pulmonary circulation should be critically evaluated as a strategy for staged surgical repair of congenital heart defects despite presumed irreversible pulmonary hypertension.     

A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: Early experience in adolescents and adults

ObjectivesDespite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was reintroduced for treatment of end-stage IPAH to permit decompression of the suprasystemic right ventricle by right to left shunting, with preservation of upper body oxygenation. The shunt has the potential to delay the need for lung transplantation and offer a treatment for those who are transplant ineligible. To optimize shunt design and avoid the potential complications of bidirectional shunting, we developed a novel approach using a unidirectional-valved shunt (UVS) in patients with IPAH with suprasystemic pulmonary arterial pressure and poor right ventricular function.MethodsA single-center retrospective review was performed of UVS cases done at Columbia University Medical Center–New York Presbyterian between November 1, 2016, and May 1, 2019.ResultsFive patients (4 female; ages 12-22 years) underwent UVS. All had suprasystemic pulmonary arterial pressure, poor right ventricular function, and World Health Organization functional class IV symptoms at baseline. All patients are alive and transplant-free at latest follow-up (range 3-33 months; median 6 ± 11 months).ConclusionsThe UVS may offer an alternative solution to lung transplantation in adolescents and young adults with IPAH. Longer-term follow-up is needed to determine the ultimate impact of unidirectional unloading of the right ventricle in these patients and to determine whether the UVS will enable a broader approach to the treatment of patients with IPAH.     

双肺移植治疗特发性肺动脉高压二例

目的 探讨双肺移植治疗特发性肺动脉高压(IPAH)的可行性及围手术期的处理方法.方法 回顾分析2例IPAH患者接受双肺移植的临床资料,2例均经右心导管术确诊为IPAH.例1为男性,17岁,体重45kg,身高165 cm,术前心功能不全Ⅳ级,肺动脉收缩压和平均压分别为110和70 mm Hg(1 mm Hg=0.133 kPa),右心房压力为18mm Hg.例2为女性,16岁,体重58 kg,身高162 cm,术前心功能不全Ⅳ级,肺动脉收缩压和平均压分别为148和72 mm Hg,右心房压力为23mm Hg.术前胸部X线片及CT显示,2例患者心脏扩大并向左偏,肺动脉增粗突起,左下肺部的血管增粗扭曲.例1和例2均在体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,先行右全肺切除,植入右供肺,后行左全肺切除,植入左供肺,完成双侧前外侧切口不横断胸骨序贯式双肺移植术.供、受者体型较为匹配,ABO血型相同或相容,供肺冷缺血时间为185～300 min.例1和例2术中ECMO支持时间分别为550和450 min,出血量分别为3000和1200 ml.结果 术后12 h患者胸部X线片和CT显示双侧移植肺清晰,心脏大小恢复正常.例1和例2分别在术后16和13 h撤离ECMO;术后第3和第4天均出现不同程度的血液动力学不稳定,发生急性左心功能衰竭,于术后第3和6天行气管切开呼吸机辅助正压通气,经强心、利尿、扩血管等治疗,分别于术后第33和12天脱离呼吸机,术后第93和32天康复出院.出院时,心脏超声检查显示,患者心脏形态及心功能明显改善,心功能达Ⅰ级.2例患者分别随访25和10个月,已恢复正常的工作和学习,生活质量良好.结论 对于终末期IPAH经内科保守治疗效果欠佳的患者行序贯式双肺移植是可行的.良好的供肺切取和保护技术、术中ECMO的支持以及术后左心功能衰竭的防治是手术成功的关键.     

Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases

BACKGROUND: Double outlet right ventricle with L-malposition of the great arteries is a rare type of double outlet right ventricle. This article reviews our experience in treating this disease surgically. METHODS: Between September 1995 and May 1999, 9 patients with double outlet right ventricle [S, D, L] underwent modified Rastelli procedure at Fu Wai Hospital. In all patients, the two great arteries originated completely from the right ventricle. The malformation was associated with subaortic conus and L-transposition of the great arteries. The ventricular septal defect (VSD) was subpulmonary in 3 patients, and non-committed in 6. All but one patient had either pulmonary valvular stenosis or subpulmonary stenosis. No patient had any type of palliative operation before. A right ventriculotomy was made to repair the VSD with a Dacron or vascular prosthesitic patch, and an intraventricular tunnel was made between the left ventricle and the aorta. The main pulmonary artery was divided and the proximal end was closed. A homograft conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. In the Rastelli procedure, the conduit is usually positioned between the right ventricular outflow tract and the pulmonary artery. RESULTS: All patients survived and recovered uneventfully. Echocardiography demonstrated that all intraventricular tunnels and valved conduits were functioning well. The results were satisfactory. CONCLUSIONS: Modified Rastelli procedure is an optimal method for surgically treating double outlet right ventricle with left-malposition of the great arteries. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.     

Single lung transplantation for pulmonary hypertension. Technical aspects and immediate hemodynamic results.

Donor availability has limited the clinical applicability of heart-lung transplantation in patients with end-stage pulmonary hypertension. Satisfaction with single lung transplantation in other patient groups prompted its extension to patients with pulmonary hypertension. Nine patients with end-stage pulmonary hypertension underwent single lung transplantation. Important technical considerations included routine use of cardiopulmonary bypass, simultaneous closure of significant associated cardiac defects (n = 4), and use of remaining thoracic donor organs in multiple recipients (total thoracic transplants from eight donors = 21). Analysis of immediate postoperative hemodynamics suggests that early relief of pulmonary hypertension and improvement in right ventricular function can be expected. There was one postoperative death. Eight patients have been discharged and are alive and well at a mean follow-up period of 1 year. All eight survivors have returned to New York Heart Association functional class I from their preoperative levels of III or IV. These results support the use of single lung transplantation as a transplant option in patients with end-stage pulmonary hypertension. The question of long-term durability remains unanswered.     

Improved results with selective management in pulmonary atresia with intact ventricular septum

OBJECTIVE: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle. METHODS: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. RESULTS: Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. CONCLUSIONS: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.