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A program to alleviate chronic, major vessel thromboembolic pulmonary hypertension by pulmonary thromboendarterectomy was initiated at this institution in 1970. Multiple evolutionary changes in the diagnostic evaluation, surgical approach, and postoperative management have been implemented over the series of 323 thromboendarterectomies performed through March 1992. A sequence of five surgeons at the University of California at San Diego have performed these procedures, with the last 150 having been performed by one surgeon. We report here the changes in surgical approach developed over the last 150 cases and the results obtained. The operation involves a median sternotomy incision, the institution of cardiopulmonary bypass, and deep hypothermia with circulatory arrest periods. Incisions are made in both pulmonary arteries into the lower lobe branches. Pulmonary thromboendarterectomy is always bilateral, with removal of both organized thrombus and an endarterectomy plane from all involved vessels. The right atrium is routinely explored for atrial septal defects. Current techniques appear to allow more thorough revascularization and shorter circulatory arrest times. The surgical mortality of 8.7% over this span is below that previously reported from this and other institutions. Among survivors, the hemodynamic and functional results have been excellent. Surgically correctable chronic thromboembolic pulmonary hypertension likely remains underdiagnosed. The diagnostic, surgical, and postoperative management evolution provided by the coordinated team involved at this institution has established that pulmonary thromboendarterectomy can be performed with an acceptable risk and good hemodynamic and symptomatic results.

HEN DISCUSSING heart failure, the left ventricle normally takes center stage, and the right ventricle (RV) can become an afterthought. However, in patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), the RV takes the lead role. These patients can present with increased right ventricular oxygen consumption with a concomitant decrease in right ventricular oxygen supply, placing them at high risk for right ventricular ischemia and failure. Fortunately, after a pulmonary thromboendarterectomy (PTE), rightheart hemodynamics greatly improve in most CTEPH patients, and right ventricular perfusion is no longer an issue. The authors, however, present a case discussing the management of a patient with severe pulmonary hypertension (PH) undergoing a PTE with residual severe PH even after their PTE.

Background Endobronchial myofibroblastic tumors are neoplasms composed of clonal populations of smooth muscle cells and a variable lymphocytic inflammatory component. They represent a challenge with respect to diagnosis, classification, and surgical resection due to their infrequent occurrence. Methods We retrospectively reviewed our experience with patients who had myofibroblastic tumors in the major airways over a 15- year period, in order to understand the incidence, natural biology, treatment, and long-term outcome of individuals with this type of neoplasm in an endobronchial location. Results Between 1995 and 2010, 11 patients (9 female, 2 male) underwent surgical resection of a myofibroblastic tumor arising within the tracheobronchial tree. The mean age was 39.6 years (range, 22.3 to 53.6 years). All patients were symptomatic, with cough and dyspnea as the most common presenting complaints. Rigid bronchoscopy with endobronchial biopsy was utilized to establish the diagnosis in 9 of 11 patients. Laser-mechanical debulking was performed to relieve airway obstruction prior to operation in 10 of 11 patients. Because of wide submucosal infiltration of the neoplasms, surgical resection for complete removal was required for all individuals. Tracheal resection was performed in 3 patients, carinal resection in 1 patient, mainstem bronchial resection in 2 patients, sleeve resection in 3 patients, bilobectomy in 1 patient, and right lower lobectomy in 1 patient. Resection with tumor-free margins was accomplished in all patients. Mean tumor size was 2.3 cm (range, 1.5 to 3.5 cm). There were no operative deaths, with all patients alive and disease-free at a mean of 6.1 ± 3.7 years. Conclusions Complete surgical resection of inflammatory myofibroblastic tumors presenting in a major airway is safe and leads to excellent survival for patients with this uncommon disease.

Pulmonary thromboendarterectomy (PTE) for the treatment of chronic thromboembolic pulmonary hypertension is an uncommon surgical procedure; however, it remains the only curative option that could provide immediate and permanent cure for this devastating disease. This condition is remarkably underdiagnosed, and as a result the procedure is uncommonly applied. Overall, patients with chronic pulmonary hypertension secondary to thromboembolic disease may present with a variety of debilitating cardiopulmonary symptoms. However, once the diagnosis is made, there is really no curative role for medical management, and medical management at best is only palliative. Surgical removal of the thromboembolic material by bilateral thromboendarterectomy is the only therapeutic option.

Summary Experience with heart and/or lung transplantation in congenital heart disease has continued to expand during the past decade. While concerns regarding the long-term outlook for transplant recipients remain, it is clear that this therapy can improve survival and quality of life in selected patients with end-stage cardiopulmonary disease when conventional surgical therapy is either not applicable or is unsuccessful. In PA-VSD the option of transplantation is available if severe pulmonary vascular or cardiac anomalies preclude conventional repair and if pulmonary vascular occlusive disease or cardiac failure develops following palliative procedures or total repair. With a number of transplantation options now available, the selection of the optimal procedure for each patient will be dependent on the cardiopulmonary anatomy and physiology.

The approach to airway management in patients undergoing lung transplantation is dependent on the type of transplant procedure (combined heart-lung, single-lung, or double-lung technique), the site of the airway anastomosis (trachea or mainstem bronchus), and whether cardiopulmonary bypass (CPB) will be used. Modifications of the original operative technique for double-lung transplantation 1,2 have been introduced recently including the use of bilateral bronchial anastomoses 3 and sequential single-lung transplantation. 4 The respective advantages of these techniques include the improved collateral blood supply at the donor bronchial anastomosic sites, thereby decreasing the risk of ischemic airway complications, and the potential elimination of the need for CPB. Specific strategies for airway management during double-lung transplantation have not been previously addressed. This report describes recent experience with a patient who underwent double-lung transplantation without CPB using a single-lumen endotracheal tube with enclosed bronchial blocker (Univent tube, Fuji Systems Corporation, Tokyo, Japan).

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by a fibrotic thrombus persisting and obliterating the lumen of pulmonary arteries; its pathogenesis remains poorly defined. This study investigates a potential contribution for progenitor cell types in the development of vascular obliteration and remodeling in CTEPH patients. Endarterectomized tissue from patients undergoing pulmonary thromboendarterectomy was collected and examined for the structure and cellular composition. Our data show an organized fibrin network structure in unresolved thromboemboli and intimal remodeling in vascular wall tissues, characterized by smooth muscle α-actin (SM-αA)-positive cell proliferation in proximal regions (adjacent to thromboemboli) and neoangiogenesis/recanalization in distal regions (downstream from thromboemboli). Cells that are positively stained with CD34 and fetal liver kinase-1 (Flk-1) (CD34+Flk-1+) were identified in both the proximal and distal vascular tissues; a subpopulation of CD34+Flk-1+CD133+cells were further identified by immunostaining. Triple-positive cells are indicative of a population of putative endothelial progenitor cells or potential colony-forming units of endothelial cells. In addition, inflammatory cells (CD45+) and collagen-secreting cells (procollagen-1+) were detected in the proximal vascular wall. Some of the CD34+cells in CTEPH cells isolated from proximal regions were also positive for SM-αA. Our data indicate that putative progenitor cell types are present in the neointima of occluded vessels of CTEPH patients. It is possible that the microenvironment provided by thromboemboli may promote these putative progenitor cells to differentiate and enhance intimal remodeling.

Notch receptor signaling is implicated in controlling smooth muscle cell proliferation and in maintaining smooth muscle cells in an undifferentiated state. Pulmonary arterial hypertension is characterized by excessive vascular resistance, smooth muscle cell proliferation in small pulmonary arteries, leading to elevation of pulmonary vascular resistance, right ventricular failure and death. Here we show that human pulmonary hypertension is characterized by overexpression of NOTCH3 in small pulmonary artery smooth muscle cells and that the severity of disease in humans and rodents correlates with the amount of NOTCH3 protein in the lung. We further show that mice with homozygous deletion of Notch3 do not develop pulmonary hypertension in response to hypoxic stimulation and that pulmonary hypertension can be successfully treated in mice by administration of N-[N-(3,5-difluorophenacetyl)-L-alanyl]-S-phenylglycine t-butyl ester (DAPT), a gamma-secretase inhibitor that blocks activation of Notch3 in smooth muscle cells. We show a mechanistic link from NOTCH3 receptor signaling through the Hairy and enhancer of Split-5 (HES-5) protein to smooth muscle cell proliferation and a shift to an undifferentiated smooth muscle cell phenotype. These results suggest that the NOTCH3-HES-5 signaling pathway is crucial for the development of pulmonary arterial hypertension and provide a target pathway for therapeutic intervention.

Prolonged air leak after thoracic procedures was successfully treated in 11 of 12 patients under local anesthesia using video thoracoscopic instillation of fibrin sealant over the site of the leak. No related complications occurred. This method should be considered an effective option for the treatment of persistent pulmonary air leaks.

Chronic thromboembolic pulmonary hypertension still remains an under-recognized and misdiagnosed condition. If left untreated, it can progress to right heart failure. Diagnosis and referral to a specialized center is paramount since surgical treatment offers a life-long cure to these patients and reversal of their right heart failure. Although new medications have been developed to treat pulmonary hypertension, they remain significantly inferior to surgical treatment in both exercise capacity and pulmonary vascular resistance improvement. The UCSD surgical classification describes the level of disease and corresponds to the difficulty of the endarterectomy. Pulmonary thromboendarterectomy remains the gold standard treatment for patients with chronic thromboembolic pulmonary hypertension. In experienced hands, pulmonary thromboendarterectomy can be performed with excellent long-term results and very low mortality.

Pulmonary endarterectomy (PTE) is the definitive treatment for chronic pulmonary hypertension resulting from thromboembolic disease. Chronic thromboembolic pulmonary hypertension is estimated to occur in approximately 4% of patients who have developed an acute pulmonary embolism, though the true prevalence is suspected to be much higher. Chronic thromboembolic pulmonary hypertension is characterized by intraluminal thrombus organization, fibrous stenosis, and vascular remodeling of pulmonary vessels. PTE is an operation considered to be a curative for this affliction and is therefore superior to transplantation. The procedure involves the removal of organized and incorporated fibrous obstructive tissue from the pulmonary arterial tree and is a true endarterectomy, not an embolectomy. Surgical outcomes with respect to functional status, quality of life, hemodynamics, right ventricular function, and gas exchange are favorable. Preoperative hemodynamic severity and site of anatomical obstruction are key predictors of postoperative outcome. This article focuses on the surgical techniques and outcomes of patients undergoing PTE for chronic thromboembolic pulmonary hypertension and discusses the currently known factors that affect survival after this operation.

The results of combined heart-lung transplantation have continued to improve following its reintroduction into clinical practice in 1981. En-bloc implantation has an apparent protective effect on subsequent tracheal anastomotic healing. Continuing developments in immunosuppression, rejection surveillance, and organ preservation will allow the procedure to fulfill its early promise in the management of end-stage lung and heart disease.

A canine double-lung allotransplantation model was developed to study the effects of 12-h static pulmonary preservation. This model has not been used extensively for such experiments but allows a detailed evaluation of the quality of preservation. An operative technique is described in which cautery dissection and preliminary devascularization of the recipient right lung allows the period of cardiopulmonary bypass to be limited and reduces postoperative bleeding. Six double-lung blocks were successfully orthotopically transplanted and recipient animals were studied for 12 h postoperatively. All animals survived the study period and had a mean PO2 (FIO2 0.4) of 141 mm Hg, 12 h following reimplantation suggesting adequate pulmonary preservation. There was no evidence of pulmonary edema at any time following implantation, although airway pressures and pulmonary vascular resistance were elevated. The role of this model in lung preservation studies is discussed.

Background Atrioventricular canal defects account for 4% of all congenital heart anomalies. They arise from failure of endocardial cushion formation, a process dependent on transition of endothelial cells into clustered mesenchymal cells in the mid–atrioventricular septum. To date, the genetic signals necessary for atrioventricular canal defects are poorly understood. We hypothesized that bone morphogenetic protein signaling in cardiac endothelial cells may be crucial to this process. Methods To study the role of bone morphogenetic protein receptors (Bmpr) in the developing heart, we created knockout mice with inactivation of Bmpr1a selectively in endocardium. Two strains of null mice were created: one with constitutive endothelial-specific knockout of Bmpr1a and one with time-inducible, endothelial-specific knockout of Bmpr1a. Embryos and animals were analyzed by microscopy, RNA in situ hybridization, and microangiography. Results Animals with null mutation of Bmpr1a in endothelium were embryonic lethal at E11.5 to 12.0 and demonstrated absence of endocardial cushion formation. Embryos failed to form atrioventricular valves and adjacent septa. Endocardial knockout of Bmpr1a did not affect development of the outflow tract or aortic arches. Using time-inducible, cell-specific knockout mice, we show that Bmpr1a has two functions in the developing atrioventricular canal: to induce endocardial endothelial-mesenchymal transition, and to pattern the septal mesenchyme into endocardial cushions. We demonstrate that these processes are temporally linked to expression of the transcription factors Id1 and Id3. Conclusions Endocardial cushion formation is dependent on cell-specific expression of Bmpr1a. Our results suggest that Bmpr1a-mediated signaling is a crucial pathway involved in pathogenesis of atrioventricular septal and valve malformations, which are among the most common congenital heart defects in humans.

Two cases of spontaneous left main coronary artery dissection are reported. This condition is rare and may present as an urgent surgical dilemma. The presentation, diagnosis, and management of primary left main coronary artery dissection are reviewed. Causative factors and underlying pathology are clarified. Prompt diagnosis and surgical intervention with temporary ventricular support is safe and effective.

Chronic thromboembolic pulmonary hypertension results from incomplete resolution of a pulmonary embolus or from recurrent pulmonary emboli. Its incidence is underappreciated, and it is currently an undertreated phenomenon. Pulmonary thromboendarterectomy is currently the safest and most effective treatment for this condition. The surgery involves midline sternotomy, profound hypothermic circulatory arrest, and complete endarterectomy of the pulmonary vascular tree. Success depends on effective coordination of multiple medical teams, including pulmonary medicine, anesthesiology, and surgery. This review, based on the past 30 years of experience at University of California San Diego Medical Center, includes information about the clinical history, diagnostic workup, anesthesia, surgical approach, and postoperative care. Outcome data are discussed, as are avenues for future research.

Objectives: To report six month outcome in patients undergoing their first pulmonary vein ablation procedure for idiopathic atrial fibrillation (AF) at a “non-pioneering” hospital. Design: Prospective observational study. Setting: Specialist electrophysiology unit at a university hospital. Patients: The first 100 consecutive patients undergoing their first pulmonary vein catheter ablation procedure for highly symptomatic, drug resistant AF in the period 1999–2002. Main outcome measures: Incidence of symptomatic or asymptomatic, Holter documented AF six months after ablation. Results: Mean patient age was 52 years (range 23–73 years), mean length of AF history 53 months (range 6–180 months), mean number of antiarrhythmic drug failures was 3 (range 1–5), and 81 were men. At the time of the ablation procedure, 64 had progressed to persistent AF and 23 had increased transverse left atrial diameter. The number of pulmonary veins ablated in each patient was one in 11, two in 45, three in 36, and four in 8. Six months after ablation, 55 patients were consistently in sinus rhythm, asymptomatic, and without any Holter evidence of AF. The chance of being in sinus rhythm was 73% (29 of 64) in those with paroxysmal as compared with only 45% (26 of 36) in those with persistent AF at the time of ablation (p = 0.01). Outcome was not influenced by patient age, length of AF history, or duration of persistent AF before ablation or to left atrial dimension. Follow up was complete and no patient has died or experienced a stroke during or after ablation; nor have any developed symptoms of late pulmonary vein stenosis. However, other complications occurred during or shortly after the procedure in 12 patients, including cardiac tamponade in six. Conclusions: In selected patients with drug resistant AF, focal pulmonary vein catheter ablation offers a realistic prospect of achieving stable sinus rhythm compared with alternatives. However, it is a complex form of ablation with a significant risk of serious complications. Although a new milestone in arrhythmia management, the optimum ablation technique is still evolving and any impact on the natural history of AF remains to be determined.

Pulmonary thromboendarterectomy, the most common surgical treatment of chronic thromboembolic pulmonary hypertension, is being performed with increasing frequency throughout the world. Massive pulmonary hemorrhage is a potentially fatal complication of this procedure. In this report of three such cases, the diagnosis, clinical course, and possible treatments are discussed. Anesthesiologists involved in the care of patients receiving pulmonary thromboendarterectomy must be aware of the possibility of this complication, as well as the various techniques available for its treatment.

Considerable progress has been made over the past decade in understanding the etiology, prevalence, natural history, and therapeutic approach to chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy is now widely recognized as the definitive treatment for chronic pulmonary hypertension resulting from thromboembolic disease. This article focuses on the surgical treatment of chronic thromboembolic pulmonary hypertension.

Recombinant cross-packaging of adeno-associated virus (AAV) genome of one serotype into other AAV serotypes has the potential to optimize tissue-specific gene transduction and expression in the heart. To evaluate the role of AAV1 to 5 virion shells on AAV2 transgene transduction, we constructed hybrid vectors in which each serotype capsid coding domain was cloned into a common vector backbone containing AAV2 replication genes. Constructs were tested for expression in: (1) adult murine heart in vivo using direct injection of virus, (2) neonatal and adult murine ventricular cardiomyocytes in vitro, and (3) adult human ventricular cardiomyocytes in vitro, using green fluorescent protein (GFP) as the measurable transgene. Serotype 1 virus demonstrated the highest transduction efficiency in adult murine cardiomyocytes both in vitro and in vivo, while serotype 2 virus had the greater transduction efficiency in neonatal cardiomyocytes in vitro. Prolonged in vivo myocardial GFP expression was observed for up to 12 months using serotype 1 and 2 vectors only. In human cardiomyocytes, serotype 1 vector was superior in transduction efficiency, followed by types 2, 5, 4, and 3. These data establish a hierarchy for efficient serotype-specific vector transduction in myocardial tissue. AAV1 serotype packaging results in more efficient transduction of genes in the murine and human adult heart, compared to other AAV serotypes. Our results suggest that adult human cardiac gene therapy may be enhanced by the use of serotype 1-specific AAV vectors.

Smooth muscle cell proliferation around small pulmonary vessels is essential to the pathogenesis of pulmonary hypertension. Here we describe a molecular mechanism and animal model for this vascular pathology. Rodents engineered to express angiopoietin 1 (Ang-1) constitutively in the lung develop severe pulmonary hypertension. These animals manifest diffuse medial thickening in small pulmonary vessels, resulting from smooth muscle cell hyperplasia. This pathology is common to all forms of human pulmonary hypertension. We demonstrate that Ang-1 stimulates pulmonary arteriolar endothelial cells through a TIE2 (receptor with tyrosine kinase activity containing IgG-like loops and epidermal growth factor homology domains) pathway to produce and secrete serotonin (5-hydroxytryptamine), a potent smooth muscle mitogen, and find that high levels of serotonin are present both in human and rodent pulmonary hypertensive lung tissue. These results suggest that pulmonary hypertensive vasculopathy occurs through an Ang-1/TIE2/serotonin paracrine pathway and imply that these signaling molecules may be targets for strategies to treat this disease.

We report the case of a 46-year-old woman who had undergone cardiac transplantation for a malignant, right ventricular sarcoma. Five years later, she experienced pulmonary hypertension and a pulmonary artery aneurysm. Medical management of the pulmonary hypertension being unsuccessful, she underwent surgical exploration of the pulmonary artery aneurysm and bilateral pulmonary endarterectomy. Intra-operative findings revealed pulmonary artery sarcoma and an unresectable pulmonary artery aneurysm.

The findings of this case report suggest that quantitative assessment of plasma neurohormones and magnetic resonance imaging functional parameters in patients with right ventricular pressure overload due to chronic pulmonary embolism might be used as indicators for right ventricular function before and after intervention. Monitoring of changes in these parameters may provide quantitative follow-up of right ventricular function in these patients. (C) 2003 by The Society of Thoracic Surgeons

Chronic thromboembolic pulmonary hypertension is associated with right ventricular dilatation, high right-sided filling pressures, and functional tricuspid regurgitation. The tricuspid regurgitation resulting from this disease has been postulated to be caused by tricuspid annular dilatation with displacement of the papillary muscles. Pulmonary endarterectomy is an operation that corrects the pulmonary hypertension resulting from chronic thromboemboli. As a result of this operation, most patients show significant improvement in tricuspid valve function. Thus, pulmonary endarterectomy is one of the few cardiac operations in which surgery remote to a valve restores valve function. This review analyzes the factors responsible for changes in tricuspid valve regurgitation after pulmonary endarterectomy and provides a strategy to predict the small subset of patients who will not show improvement in tricuspid valve function after this operation.

Because pulmonary thromboendarterectomy (PTE) can result in an immediate reduction in pulmonary artery (PA) pressure, we sought to evaluate the effect of PTE on severe tricuspid regurgitation (TR) without tricuspid annuloplasty.Few data exist regarding the frequency and magnitude of functional TR improvement after reduction in PA pressure.We identified 27 patients with severe TR, defined by a regurgitant index (RI)33%, who underwent PTE. The RI, tricuspid annular diameter (TAD), apical displacement of leaflet coaptation, and estimated PA systolic pressure were determined on pre- and post-PTE echocardiograms. Patients were stratified based on resolution (RIor =33%) or persistence (RI33%) of severe TR.Comparing pre- and post-PTE echocardiography results, severe TR resolved in 19 of 27 (70%) patients. This group had a more effective PA systolic pressure reduction after PTE (49 +/- 20 mm Hg vs. 32 +/- 16 mm Hg by echocardiography, p = 0.075, and 37 +/- 16 mm Hg vs. 16 +/- 13 mm Hg by catheter measurement, p = 0.004). No difference was observed in TAD, apical displacement of the tricuspid valve, or other features compared with the group with persistent severe TR. There was a trend toward longer hospital stays in the group with persistent severe TR (19 +/- 15 days vs. 14 +/- 9 days; p = 0.55).After significant PA pressure reduction by PTE, severe functional TR with a dilated annulus may improve without annuloplasty despite dilated tricuspid annulus diameters.

Differences between bilevel ventilators used for noninvasive intermittent positive pressure ventilation (NIPPV) have been demonstrated during bench testing. However, there are no clinical studies comparing these machines. The authors have previously shown that the Quantum pressure support ventilator and Sullivan variable positive airway pressure II ST differ in performance during bench testing. To examine the clinical significance of this, these two machines were compared in the overnight treatment of subjects with chronic respiratory failure. Ten clinically-stable subjects with thoracic scoliosis were recruited. The subjects were already established on NIPPV, but none were using either of the ventilators to be tested. After familiarisation, the patients used the two ventilators in random order on consecutive nights. Peripheral oxygen saturation and transcutaneous carbon dioxide tension (Pt,CO2) were measured continuously, and sleep was recorded using polysomnography. There were no significant differences in arterial oxygen saturation, Pt,CO2 or sleep duration and quality between the two nights. Despite previously illustrated variation in laboratory performance, no differences were seen between the two ventilators when comparing overnight gas exchange and sleep in vivo. Further study is required to evaluate the significance of the differences found during bench testing in the clinical setting.

Transplant recipients living in endemic areas are at high risk of aerosol-transmitted fungal infections because of environmental exposure while on immunosuppressive drugs, as well as reactivation of latent infection from either the patient's or the donor's organs. The latter may account for early development of coccidioidomycosis after transplantation. We describe a case of pulmonary coccidioidomycosis in a lung transplant recipient who acquired the infection from the donor lung and presented with fulminant pneumonia in the immediate postoperative period.

Optimal timing for consideration of lung transplantation remains unknown. This study examined survival in patients with end-stage lung disease awaiting transplantation. Primary disease group and relevant indicators were evaluated. Ninety-three patients who met selection criteria for lung transplantation were included in this retrospective review. Of this total, 31% underwent transplantation, 38% remain waiting, and 31% died. Results demonstrate that the six-month actuarial survival rate was 89% for Eisenmenger's syndrome, 81% for emphysema, 74% for cystic fibrosis, 60% for primary pulmonary hypertension, and 38% for interstitial lung disease. Parameters found to be significant included a higher mean right atrial pressure in primary pulmonary hypertension patients who died awaiting transplantation, and lower forced expiratory volume in one second and forced vital capacity measurements in cystic fibrosis patients who died awaiting transplantation. We conclude that primary disease significantly affects survival in candidates awaiting transplantation. Reliable indicators predictive of survival are not available. Earlier referral for consideration of lung transplantation is recommended.

J Thorac Cardiovasc Surg 2001;121:1213-5

Recurrent symptomatic pulmonary hypertension is uncommon after primary pulmonary thromboendarterectomy (PTE). We reviewed our experience with patients undergoing repeat PTE to determine the risk factors for recurrent disease, and the selection criteria, relative risks, and functional outcomes of reoperative PTE.Since 1990, 13 of 870 (1.5%) patients underwent reoperative PTE at our institution. These 7 men and 6 women (mean age 38.6 years) were contrasted with the most recent 225 patients (111 men, 114 women, mean age 52.7 years) who underwent primary PTE for whom complete hemodynamic data are available. The preoperative evaluation of all patients was similar. Pulmonary hemodynamic data and outcome measures were compared between groups.Of 13 reoperated patients: 69% (9/13) had their primary operation at another institution, 54% (7/13) initially underwent unilateral PTE, 38% (5/13) had identifiable coagulation disorders, 38% (5/13) had ineffective caval filtration, 31% (4/13) had suboptimal anticoagulation management, and 31% (4/13) had complete unilateral pulmonary artery obstruction. The mean interval to reoperation was 5.2 years (range 0.7 to 10.9 years). All control patients underwent bilateral PTE using hypothermic circulatory arrest. Operative mortality was 7.7% (1/13) with reoperation vs 8.4% (19/225) in controls. No difference (p = NS) was observed between groups in the preoperative pulmonary artery pressure (PAP) or pulmonary vascular resistance; however, the control group had a significantly (p0.05) greater reduction in the postoperative PAP (46/19, mean 28 mm Hg vs 59/23, mean 35 mm Hg) and PVR (271 +/- 172 vs 399 +/- 154 dynes/s/cm(-5)) compared with the redo group. No substantial difference in morbidity or functional outcomes was observed between groups.Reoperative PTE can be performed with a perioperative risk comparable with primary PTE, although the improvement in pulmonary hemodynamics is not as favorable. Bilateral primary operation, effective caval filtration, and vigilant anticoagulant management would prevent the need for most reoperative PTEs.

Long waiting times for lung transplantation have limited the survival of patients with advanced pulmonary hypertension. Atrial septostomy has been used in this group of patients in an attempt to prolong survival. We evaluated the results of atrial septostomy in 12 patients using the static graded balloon dilation technique. Between December 1990 and May 1998, 10 women and 2 men (ages 13 to 56 years, mean 37 years) underwent atrial septostomy. Nine patients had primary and 3 patients had secondary pulmonary hypertension. Five patients deteriorated despite long-term intravenous prostacyclin infusions. The atrial septum was crossed with a Brockenbrough needle, followed by an 0.035-J exchange wire and progressively larger catheter balloons for atrial septal dilation, until systemic oxygen saturation decreased 5% to 10%. An atrial septal defect was successfully created in each patient. The mean right atrial pressure decreased from 23 to 18 mm Hg and the mean systemic oxygen saturation decreased from 93% to 85%. The mean cardiac index increased from 1.7 to 2.1 L/min/m 2 and the mean systemic oxygen transport increased from 268 to 317 ml/min/m 2 . Complications occurred in 3 patients: transient hypotension during transesophageal echocardiography, a femoral pseudoaneurysm, and a femoral arteriovenous fistula. After septostomy, 6 patients had clinical improvement (resolution of ascites, edema, and no further episodes of syncope); 5 of these 6 patients underwent lung transplantation a mean of 6.1 months after septostomy. Six patients did not have clinical improvement after septostomy. Atrial septostomy improves the hemodynamic status and may be useful as a bridge to lung transplantation in selected patients with pulmonary hypertension.

This study evaluated long-term outcome of pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Survival, functional status, quality of life, health care utilization, and relationships between these parameters and postoperative pulmonary hemodynamics were assessed. Questionnaires were mailed to 420 patients who were more than 1 yr post-PTE; 308 responded (mean age, 56 yr [range, 19-89 yr]; mean years since PTE, 3.3 [range, 1- 16]). Survival after PTE was 75% at > 6 yr. After surgery, symptoms were markedly reduced. Median distance walked was 5,280 ft; 56 patients could walk "indefinitely." Of the working population, 62% of patients unemployed before PTE returned to work. Post-PTE patients scored several quality of life components of the Rand SF-36 slightly lower than reported normals but significantly higher than did pre-PTE patients. Ten percent of patients used oxygen. Ninety-three percent were in NYHA Class I or II. Disease-related hospitalizations/ER visits were minimal. A relationship was shown between 48 h postoperative pulmonary vascular resistance (PVR) and walking and stair-climbing ability, NYHA class, dyspnea scores, and the physical function and general health quality of life components. These data indicate that PTE offers most CTEPH patients substantial improvement in survival, function, and quality of life, with minimal disease-related health care utilization.

Intrapericardial teratoma is a rare, often benign congenital tumor, which frequently leads to respiratory distress and pericardial tamponade shortly after birth. Surgical excision is not only curative but potentially lifesaving, because these lesions often become fatal if not promptly excised. We present a case of one of two twins diagnosed with a large intrapericardial teratoma. The management of this case was complicated by the necessity to consider the well-being of the other (normal) twin in planning the timing of delivery and operation. After the twins were delivered by cesarean section in one operating room, the mass was excised from the infant in an adjoining operating room with cardiopulmonary bypass standby.

The ideal prosthetic valve would have excellent hemodynamics (similar to a normal human valve in the same position), last a lifetime, be free of structural dysfunction or breakdown, and require no particular medical therapy such as anticoagulation. Needless to say, such a valve is yet to be

Unlike infections of the native heart valve, which are often cured by appropriate antibiotic therapy, prosthetic valve endocarditis usually necessitates an operative ap­ proach for adequate treatment. Prosthetic valve endocarditis is associated with a greater mortality and morbidity than is native valve endocarditis, and this relationship is attributable to the increased likelihood of annular destruction and the increased complexity of surgical repair. The risk of pros­ thetic valvular disease is greatest early after operation, and affected patients are also more likely to have hospital-based virulent and destructive organisms, which are less respon­ sive to antibiotic therapy. Fortunately, the frequency of occurrence of prosthetic valvular endocarditis is only ap­ proximately 2 to 4%.4 Although endocarditis remains a devastating and often lethal condition, there have been recent advances in its diag­ nosis and therapy. The evolution of transesophageal echo­ cardiography has led to its increasing application in the assessment of cardiac valvular abnormalities. Operative treatment has improved with a firmer understanding of the need for the complete removal of all devitalized and infected tissue, if necessary in combination with major surgical re­ construction, and an increased use of homograft tissue valves. Echocardiography.-Echocardiography is helpful in de­ tecting various cardiac lesions in endocarditis, including valvular regurgitation, annular ring abscesses, and vegeta­ tions. Transesophageal is better than transthoracic echo­ cardiography in finding evidence of vegetations, quantifying valvular dysfunction, and diagnosing the spread of infec­ tion.' This advantage is especially noted in prosthetic valve endocarditis, in which shielding by the prosthesis may pre­ vent adequate transthoracic diagnosis of vegetations or ab­ scess cavities. What is the significance of finding vegetations on echocardiography? Although seemingly large and loose vegetations may be a daunting phenomenon when discov­ ered echocardiographically, no firm evidence suggests that this appearance, in itself, should trigger surgical interven­ tion. As pointed out in the article by Mullany and associates, although one series showed that mitral vegetations or large vegetations were more likely than others to embolize," this finding did not necessarily prevail in treated patients at the Mayo Clinic," Of interest, antibiotic treatment rather than anticoagulation has been noted to inhibit embolization." Nevertheless, emboli are common (25% of patients in the current series) and usually involve the brain. The frequency of emboli is the same with both native and prosthetic valve endocarditis. Antibiotic treatment should be with bacteri