Tetralogy of Fallot with a Single Pulmonary Artery: Operative Repair

Surgical repair was performed on 8 patients with tetralogy of Fallot and congenital or acquired absence of the left pulmonary artery. Prior palliative systemic-pulmonary artery shunts had been performed in the 5 patients with acquired absence of the pulmonary artery, and in none was repair of the damaged pulmonary artery possible. A valve-containing conduit (Hancock) was used in each patient to prevent pulmonary valvular regurgitation during the early postoperative period. All patients survived operation and have exhibited marked symptomatic and hemodynamic improvement. This contrasts with the poor results of corrective operation previously reported in patients in this diagnostic category. We believe that the present improved results are due in large part to the valved conduit, and we therefore recommend its use during repair in children or adults with tetralogy and a single pulmonary artery.     

Analysis of factors leading to posttraumatic pulmonary insufficiency

Clinical and pathological data on all trauma patients (113) on whom a postmortem examination was performed between January, 1975, and July, 1976, were analyzed to determine the factors leading to posttraumatic pulmonary insufficiency (PTPI) defined as treatment-resistant lung failure. Fifty-nine patients died within 3 days of injury; 28 had suffered hemorrhagic shock and received massive fluid therapy (>10 liters). While mechanical ventilation was used more often in those patients suffering from shock (p < 0.01), only 3 died with lung failure. There was little difference in lung weight and histopathological findings between patients suffering from shock and patients who died of head injury (who received little intravenous resuscitation). Interstitial edema and atelectasis were present in both groups. The 3 patients with early PTPI had hemorrhagic consolidation and alveolar edema. Of the 54 patients who died after 72 hours, 32 had PTPI. Shock and its treatment were not associated with late PTPI (p > 0.08). The three factors associated with late PTPI were sepsis (p < 0.001), pulmonary infection (p < 0.001), and remote infection (p < 0.001). Thirty of 32 patients had pneumonia with klebsiella or pseudomonas or both.Most patients suffering from shock do not have pulmonary failure, and modern ventilatory support is adequate treatment for the usually mild respiratory dysfunction they experience. The combination of mild pulmonary edema and mechanical ventilation may lead to gram-negative pulmonary infection manifest as PTPI.     

Heart-Lung Transplantation for Irreversible Pulmonary Hypertension

Combined heart and lung transplantation was carried out in 17 patients at Stanford University between March, 1981, and December, 1983. The recipients were between 22 and 45 years old. All patients had end-stage pulmonary hypertension; 10 had Eisenmenger's syndrome and the remaining 7, primary pulmonary hypertension. Five patients died within the first few postoperative weeks. The remainder are well between four weeks and 33 months from operation.The immunosuppressive protocol has consisted of cyclosporine with an initial course of rabbit antithymocyte globulin. Azathioprine also was given for the first two weeks and then was replaced with prednisone. Rejection, as diagnosed by cardiac biopsy, was treated with high doses of methylprednisolone. Modifications of technique that have developed include the removal of the recipient heart and lungs separately, and preservation of the lungs with a modified Collins' solution instead of a cardioplegic solution.Rejection occurred in 6 of the 12 survivors. Infections developed in 9 patients, but only one resulted in a fatal outcome (Legionella). Thus, the results of clinical heart-lung transplantation have been considerably superior to clinical efforts in lung transplantation. It is suggested that the combined operation is preferable for the following reasons: (1) all diseased tissue is removed, thus eliminating recurrent infection and ventilation/perfusion disparity; (2) transplantation of the entire heart-lung block preserves coronary–bronchial vascular anastomoses and makes airway dehiscence less likely; and (3) to date, diagnosis of rejection by cardiac biopsy has appeared to be a satisfactory method of diagnosing and treating pulmonary rejection. Cardiopulmonary transplantation represents a viable therapeutic approach for patients with end-stage pulmonary hypertension with or without associated congenital heart disease.     

Pulmonary aspergilloma. A report of its occurrence in 2 patients with cyanotic heart disease.

This is an account of our experience with 2 patients with congenital cyanotic heart disease who developed pulmonary aspergillomas. Neither patient had a demonstrable cavity in the lung before the appearance of the fungus ball. Both patients had pulmonary artery-to-superior vena cava shunts, but the aspergilloma was in the ipsilateral lung in one and the contralateral lung in the other. Repeated life-threatening hemoptyses made excision of the lung containing the cavity and fungus ball necessary in both patients.     

Successful early surgical recruitment of the congenitally disconnected pulmonary artery

BACKGROUND: Our purpose was to document our experience with early recruitment of congenitally disconnected pulmonary arteries and to assess subsequent pulmonary artery growth and function. METHODS: Patients born in the 10-year period from 1989 to 1999 with a disconnected pulmonary artery diagnosed in infancy and treated in our unit were studied. To be included patients had nonconfluent pulmonary arteries with one or both completely disconnected from the main pulmonary artery. This series did not include patients with acquired stenosis causing occlusion of a pulmonary artery. Echocardiography, cardiac catheterization, MRI, lung perfusion scans, and intraoperative assessment were used to gauge pulmonary artery growth and function. RESULTS: Seven patients with a disconnected pulmonary artery associated with intracardiac conotruncal congenital cardiac disease underwent successful early surgical recruitment of the affected pulmonary artery at 3 months of age or younger. Median follow-up from date of first operation was 4.2 years (range, 1.6 to 13.4). All 7 patients had postrecruitment lung perfusion scans showing a mean of 44% (range, 27% to 78%) of total pulmonary flow through the affected lung. Significant growth in the diameter of the recruited native pulmonary artery was demonstrated in all patients. There were no deaths reported in our series to date. CONCLUSIONS: The rare possibility of a congenitally disconnected pulmonary artery needs to be considered in all patients with a conotruncal cardiac anomaly. To facilitate surgical correction, ensure subsequent growth of the pulmonary artery, and optimize associated lung development, early diagnosis and surgical recruitment is recommended.     

Outcome after reconstruction of discontinuous pulmonary arteries

OBJECTIVE: This study was undertaken to determine outcomes of and optimal treatment strategies for reconstruction of congenital or acquired discontinuity of branch pulmonary arteries. METHODS: Between 1985 and 2000 pulmonary artery continuity was established in 102 patients with discontinuous central pulmonary arteries and normal peripheral arborization. Data were obtained retrospectively. RESULTS: Techniques to connect both pulmonary arteries included direct pulmonary artery-pulmonary artery anastomosis (n = 33), tube graft interposition (n = 47), or pulmonary arterial implantation in right ventricular-pulmonary arterial conduits (n = 22). Among patients with biventricular repair (n = 66), survival was 85% +/- 8% at 5 years, and freedom from surgical or interventional pulmonary arterioplasty was 31% +/- 11%. At most recent follow-up, mean branch pulmonary arterial z scores were -0.5 +/- 1.6 (right pulmonary artery) and -1.4 +/- 1.3 (left pulmonary artery). Mean right to left ventricular pressure ratio was 0.61 +/- 0.26, and this value was more than 0.75 in 13 of 58 cases. Fifteen of 51 had a lung perfusion mismatch of more than 75:25, and in 9 of 58 one branch pulmonary artery was occluded. Twenty-two patients who underwent primary establishment of antegrade pulmonary artery flow without previous shunt procedures had comparable survival and reintervention rates, with a tendency toward higher pulmonary arterial z scores and lower right to left ventricular pressure ratios. Among patients with single-ventricle repair (n = 33), 5-year survival was 93% +/- 8% and freedom from pulmonary arterioplasty was 39% +/- 9%. Ten of 19 patients had a lung perfusion mismatch, and one branch pulmonary artery was occluded in 4 of 31. Overall, a direct pulmonary artery anastomosis was associated with better survival (P =.006). The presence of aortopulmonary collaterals was a risk factor for pulmonary artery occlusion (P =.03). CONCLUSION: Good survival can be achieved for patients with pulmonary artery discontinuity, but this requires frequent reinterventions. Direct pulmonary artery- pulmonary artery anastomoses and control of all collateral vessels may further improve outcome.     

Surgical palliation of single ventricle with pulmonary stenosis

Two patients aged 9 and 18 years underwent palliative correction of single ventricle with pulmonary stenosis by insertion of a fascia lata composite graft between the anterior aspect of the ventricle and the main pulmonary artery. Both patients had satisfactory hemodynamic states postoperatively, but 1 died of subarachnoid hemorrhage and the other from generalized infection. The surgical technique and the pathology as seen at operation and at postmortem examination are described. The potential merits of this type of palliation are discussed.     

Therapeutic considerations in congenital absence of the right pulmonary artery. Use of internal mammary artery as a preparatory shunt

Congenital unilateral absence of a pulmonary artery is rare. When present, it is usually associated with other cardiac malformations. In this communication an infant with coarctation of the aorta, ventricular septal defect and absent right pulmonary artery is reported. After correction of the first two defects, due to persistent respiratory difficulty, the infant had exploration of the right pulmonary hilum in hopes of finding a pulmonary artery remnant and re-establishing blood flow to that lung. A small hilar vessel supplying all three lobes of the right lung was found and as a first stage, to enhance pulmonary arterial growth, a palliative systemic to pulmonary artery shunt was performed utilizing a large internal mammary artery. This is the first report of a case in which the internal mammary artery is used in an infant to establish systemic to pulmonary arterial flow in congenital unilateral absence of a pulmonary artery.     

Aneurysm of main pulmonary artery in a neonate with airway obstruction and heart failure: long-term survival after pulmonary artery aneurysmectomy and patent ductus arteriosus ligation

A neonate was seen with complete atelectasis of the left lung secondary to compression of the left main bronchus by a congenital aneurysmal main pulmonary artery. Operation consisted of pulmonary artery aneurysmectomy and ligation of an associated patent ductus arteriosus. Follow-up (3 years after operation) demonstrated complete resolution of the atelectasis and congestive heart failure.     

儿童单侧肺动脉缺如X线平片及CT表现

目的探讨先天性单侧肺动脉缺如(UAPA)的X线平片及CT表现。方法回顾性分析14例UAPA患儿的影像学资料,观察其X线平片及CT平扫特征。结果 X线平片:患侧肺体积减小9例(9/14,64.29%),透光度减低7例(7/14,50.00%),肺纹理较健侧稀疏9例(9/14,64.29%),合并肺部感染4例(4/14,28.57%);CT平扫:患侧肺体积减小12例(12/14,85.71%),透光度减低7例(7/14,50.00%),肺纹理纤细9例(9/14,64.29%),肺动脉主干缺如1例(1/14,7.14%),合并肺实变、间质改变8例(8/14,57.14%),合并肺内囊变3例(3/14,21.43%)。结论 UAPA的X线平片和CT平扫图像具有一定特征,对诊断UAPA具有重要提示作用。     

Bullet Emboli to the Pulmonary Artery: A Report of 2 Patients and Review of the Literature

Bullet embolization to the pulmonary artery is a rare event. The purpose of this study is to report our experience with 2 patients and to review the 15 patients reported in the literature, with special emphasis on a rather peculiar complication that has occurred in 4 of the 9 patients who underwent bullet embolectomy: dislodgment of the missile during the surgical procedure and migration to the down-side lung, for which a second thoracotomy was required in 3 of those patients.The usual untoward effects of foreign bodies in the vascular system were seen in this series: embolization with thrombosis, sepsis, erosion and hemorrhage, and vascular occlusion with infarction.This review suggests that operative removal of a bullet in the pulmonary artery is necessary. The operation is safe and uncomplicated if precautions are taken to prevent the missile from migrating during manipulation of the lung.     

Double-outlet left ventricle

Origin of both great vessels from the left ventricle is an uncommon congenital cardiac anomaly. When there is an associated ventricular septal defect and pulmonary stenosis, the preoperative diagnosis has usually been that of tetralogy of Fallot. Operative repair was accomplished successfully in a 12-year-old boy with such a combination of defects. This involved patch closure of the ventricular septal defect, interruption of blood flow from the left ventricle to the pulmonary artery by suture closure of the pulmonary valve and annulus, and restoration of blood flow to the lung by using a Dacron tube conduit between the right ventricle and the distal main pulmonary artery. This represents the third successful operative repair of double-outlet left ventricle.     

Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration.

Recently we saw 9 infants with life-threatening respiratory distress. Four patients had bronchogenic cyst, 2 had cystic adenomatoid malformation, and 9 had congenital lobar emphysema. Another group of 14 older children had recurrent infection and hemodynamic abnormalities, which responded to operative intervention. Each child required an appropriate resection following definitive diagnosis. These lesions represent a spectrum of closely related anomalies that arise during an early stage of embryonic lung bud maturation. Bronchoscopy is rarely useful, but special roentgenographic studies, including perfusion scans and arteriography, are usually diagnostic. Our operative experience is used to emphasize the urgency of precise diagnosis and surgical management of this poorly recognized clinical syndrome.     

Analysis of Various Treatments for Pulmonary Aspergillomas

The objective of this study was to analyze the results of various treatments for pulmonary aspergilloma and to determine the surgical indications. A total of 41 patients with pulmonary aspergilloma hospitalized at the National Minamifukuoka Chest Hospital between 1973 and 1999 were analyzed with regard to their response to treatment and long-term prognosis. Five asymptomatic patients who were untreated demonstrated no change in the clinical status of aspergilloma. Analysis of the short-term response revealed surgery to be the most effective treatment. Systemic and intracavitary injections of antifungal drugs were not definitely effective, although they had a positive effect in some patients. A survival analysis revealed that all eight patients who underwent surgery are still alive. Using the Cox proportional hazard model, it was found that the favorable prognostic factors were the absence of symptoms, the absence of a superimposed bacterial infection, and surgery. There were ten deaths: three from lung cancer and seven related to uncontrollable superimposed bacterial infections, often resulting in hemoptysis. We conclude that patients with asymptomatic pulmonary aspergillomas should be clinically observed for signs of the development of lung cancer, but do not require active treatment. On the other hand, patients who are symptomatic and have uncontrollable superimposed bacterial infection will benefit from surgery. Received: August 24, 2000 / Accepted: March 6, 2001     

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.     

Asynchronous rejection of heart and lungs following cardiopulmonary transplantation

Eighteen patients have received 19 combined heart-lung allografts since March, 1982. During the maturation of our program of heart-lung transplantation, we have learned that isolated rejection of the lung can occur frequently and that exclusive dependence on the cardiac biopsy can be misleading. Of the 18 patients who received allografts, 10 are the basis for this report. The other patients were excluded because of death from excessive bleeding (1), inadequate lung preservation (2), an inability to differentiate rejection from infection (3), or an absence of rejection of either the heart or the lungs (2). Rejection of the lung was suggested, in the absence of clinical evidence of infection, by the radiographic appearance of a diffuse pulmonary infiltrate. It was confirmed by a prompt response to augmentation of maintenance immunosuppression with an intravenous pulse of methylprednisolone. The presence or absence of cardiac rejection was determined by the standard endomyocardial biopsy. Direct biopsy of the involved lung through a thoracotomy was performed in 4 patients so that a definitive histological diagnosis of rejection would reinforce the anticipated clinical diagnosis. The clinical course in 6 of the 10 patients plus the results of the open lung biopsy in 3 of them suggest that isolated rejection of the lung developed in the absence of cardiac findings. Patients responded within 12 to 24 hours to augmented immunosuppression with a dramatic improvement in the abnormal chest radiograph. In all 10 patients, either isolated lung or synchronous heart and lung rejection episodes were confined to the first six weeks after operation unless a severe alteration in the immunosuppression was made (2 patients).(ABSTRACT TRUNCATED AT 250 WORDS)     

先天性肺囊性腺瘤样畸形的诊断和治疗

目的 总结小儿先天性肺囊性腺瘤样畸形的诊断及手术治疗，提高对该病的认识。方法 对24例术前均摄胸部X线平片、CT并经手术、病理证实的先天性肺囊性腺瘤样畸形病儿的临床资料进行回顾性总结。其中男13例，女11例，平均年龄4．75岁，主要症状是呼吸道感染及呼吸困难，病史ld至8年。结果 影像学术前确诊率79．17％。24例行开胸术，证实病变均为单侧，侵犯1个肺叶22例，其中左叶15例、右叶7例；侵犯2个以上肺叶者2例。14例与支气管无交通，l例合并胸主动脉的异常血供，l例合并巨大实性肿物。20例行肺叶切除、3例行肺段切除、l例因胸膜肺母细胞瘤行全肺切除。全组无术后并发症。按Stocker分型：I型13例，Ⅱ型9例，IⅡ型2例。结论 影像学检查是诊断先天性肺囊性腺瘤样畸形的主要方法。对有症状者，手术切除病变侵犯的肺组织是治疗该病安全、有效的方法。     

Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia

Purpose

This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI).

Methods

A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten patients with systemic/suprasystemic and 9 patients with subsystemic pulmonary hypertension at 3 weeks of age were identified. Diameters of the right pulmonary artery, left pulmonary artery (LPA_d), aorta, and the length of vermis of the cerebellum were obtained from prenatal magnetic resonance imaging to calculate the PPHI [=(LPA_d/length of vermis of the cerebellum) × 10] and the modified McGoon index (MGI) [=(diameter of the right pulmonary artery + LPA_d)/diameter of aorta]. Prenatal pulmonary hypertension index and MGI were compared with lung-to-head ratio, percent predicted lung volume, and total lung volume for pulmonary hypertension and survival.

Results

The PPHI and MGI had a significant, negative correlation with pulmonary hypertension (r = −0.61, P = .005, and r = −0.72, P < .005, respectively). The PPHI and MGI are significantly lower in the systemic/suprasystemic PAH group compared with the subsystemic PAH group (1.11 ± 0.32 versus 1.63 ± 0.28, P = .004, and 0.71 ± 0.15 versus 1.05 ± 0.11, P < .001, respectively). There were no significant differences between the groups comparing the lung-to-head ratio, percent predicted lung volume, and total lung volume.

Conclusion

Both PPHI and MGI accurately predict the severity of postnatal PAH in isolated left CDH.     

Experimentally reversed pulmonary blood flow. Implications for the surgical management of complex forms of congenital heart disease.

Blood flow to the right lung was reversed in 28 dogs, 14 of which survived more than 1 month. Four successful long-term survivors (1 years to 4 years 7 months) were repeatedly studied by cardiac catheterization, angiography, and ventilation/perfusion scans as well as hemodynamically when they were killed. Hemodynamics and gas transfer were normal up to 4 1/2 years after the procedure, and the histology of the right lung was essentially normal. The application of reversed pulmonary blood flow is proposed in the surgical management of forms of congenital heart disease in which the right ventricle is diminutive. The procedure consists of repartitioning of the atria to allow venous blood to flow into the lung through the pulmonary veins and exit to the left atrium through the pulmonary artery.     

Clinical spectrum of congenital cystic disease of the lung in children.

OBJECTIVES: Congenital cystic lesions of the lung are uncommon but share similar embryologic and clinical characteristics. The purpose of this study is to review our institutional experience of congenital cystic lung disease, emphasizing the clinical spectrum of the disease related to age, and present some cases with unusual clinical manifestations. PATIENTS: Between 1962 and 1996, 26 patients (9 females and 17 males) under 15 years old underwent evaluation and surgical treatment for congenital cystic lung disease. Seven patients were under 1 year old, and 19 were in over 1 year old. There were 13 bronchogenic pulmonary cysts, 6 pulmonary sequestrations, 4 congenital cystic adenomatoid malformations (CCAM), and 3 congenital lobar emphysemas. RESULTS: All patients under 1 year old showed respiratory distress with mediastinal shift but no episodes of infection. In contrast, 13 of the 19 patients over 1 year old had symptoms of recurrent infection without respiratory distress. Five patients over 1 year old were entirely asymtomatic from birth. There were significant differences (P < 0.05) in the frequencies of respiratory distress and infection between the two groups (chi2-test). Lobectomy was performed in 21 patients, excision in 3 patients, segmentectomy in one patient, and exploration in one patient. There was no incident of postoperative mortality or morbidity except for one patient with CCAM complicated by reexpansion lung edema. Twenty-one patients at long-term follow-up from 2 to 30 years after surgery are doing well with no subsequent limitation of physical activities due to lung resection. CONCLUSIONS: In patients under 1 year old, cystic lesions were discovered by respiratory distress; and in patients over 1 year old signs of infection were the most important clinical features. Early recognition of these relatively rare congenital cystic lung lesions would lead to the immediate, proper surgical intervention.