Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease.

Five patients with partial anomalous pulmonary venous drainage with intact atrial septum are described. In two patients, pulmonary arterial hypertension and pulmonary vascular disease developed. Both had one or more right pulmonary veins draining anomalously to the right superior vena cava. It is postulated that a combination of increased pulmonary blood flow and reflex pulmonary vascular changes contributes to the production of pulmonary vascular obstructive disease.

Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease.

The condition of patients with transposition of the great arteries, intact ventricular septum and severe pulmonary vascular disease is inoperable with present techniques. In a series of 260 surgically treated patients with transposition of the great arteries and intact ventricular spetum, 5 had severely increased pulmonary vascular resistance, and all 5 died; postmortem examination confirmed the presence of severe pulmonary vascular disease. The concept of the "palliative Mustard" procedure was applied in two children with transposition of the great arteries, intact ventricular spetum and pulmonary vascular disease who underwent the Mustard operation with creation of a ventricular septal defect. The postoperative course was uneventful in both patients. Thirteen and 5 months, respectively, after operation, both are physically active and have respective arterial oxygen saturation levels of 94 and 92 percent.

The treatment of pulmonary heart failure.

Treatment of acute pulmonary heart failure is usually achieved with rest, relief of alveolar hypoxia and diuretics. Apart from thromboembolism, emphasis is placed on prophylaxis and the active management of the pulmonary dysfunction, including regular functional assessment to guide therapy directed particularly at the relief of alveolar hypoxia.

Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis.

Pulmonary alveolar microlithiasis (PAM) is characterized by deposition of calcium phosphate within the alveolar airspaces. There is currently no effective medical therapy and affected individuals may progress to end-stage lung disease requiring transplantation. Two patients with PAM underwent bilateral sequential lung transplantation. This study reviews the clinical manifestations of PAM and discusses the particular difficulties that may be encountered in the use of lung transplantation as treatment for this uncommon disease. Also addressed is the question of recurrence in the allograft.

Technical aspects of adult lung transplantation.

This article describes the authors' current technical preferences for the performance of isolated single lung transplantation and bilateral sequential single lung transplantation. The current techniques are the result of lessons learned in the performance of over 400 lung transplant operations at Washington University School of Medicine since 1987.

Cardiac and pulmonary failure secondary to adenotonsillar hypertrophy.

For over 15 years, upper respiratory tract obstruction due to adenotonsillar hypertrophy has been known to cause hypoxia, hypercapnia, increased pulmonary vascular resistance and thereby cor pulmonale and congestive heart failure. This is now an uncommon but not rare entity and three recent cases prompted this report. The typical patient is dyspneic with retractions, cyanosis, occasional periods of apnea and somnolence. Edema and hepatomegaly and at times splenomegaly are common. X-rays show cardiomegaly, which on electrocardiogram is found to involved mainly the right ventricle. The strict definition of cor pulmonale is right ventricular hypertrophy secondary to lung disease or abnormal pulmonary function, a definition that may logically be stretched to include abnormal respiratory function secondary to upper airway pathology. The mechanisms by which this occurs are generally agreed upon. Hypoxia has been demonstrated to cause pulmonary vasoconstriction. Acidosis and hypercapnia are thought by some to have the same effect. Pressure across the pulmonary vascular bed is also increased, as predicted by Poiseuille's law, by the high rate of blood flow required to maintain tissue oxygenation with poorly oxygenated blood. Conditions producing hypoxia of hypercapnia or both lead to hypertrophy and eventually to dilatation of the right ventricle. Three cases of children who underwent cardiac catheterization while suffering from cor pulmonale due to adenotonsillar hypertrophy are reported. Right ventricular pressure averaged 44/5, PAO2 72, pH 7.32, and PACO2 52. All were clinically improved following adenotonsillectomy. Cardiac catheterization was repeated in one case, with right ventricular pressure dropping from 44/5 to 21/2, pulmonary vascular resistance from eight units to three, and PACO2 from 62 to 44.

Coronary to bronchial artery anastomosis in patients with noncyanotic cardiopulmonary disease: report of seven cases.

An angiographically visible coronary to bronchial artery anastomosis was found in seven (0.12%) of 6045 patients with noncyanotic cardiopulmonary disease who underwent coronary angiography between 1989 and 1995. Aortitis syndrome was associated with four patients, whereas pulmonary embolism, aortic regurgitation and vasospastic angina were the diagnoses in the others. Coronary stenotic lesions were not observed in any patients. In five of six patients who underwent pulmonary perfusion scintigraphy, perfusion defect was observed in the area supplied by the bronchial artery, which had the anastomosis to the coronary artery. In each patient this anastomosis seemed to function as collateral circulation, compensating for decreased perfusion in either the lung or the heart. When coronary to bronchial artery anastomosis is found, ischemic conditions in either the lung or the heart are likely.

Paradoxical embolism. An old but, paradoxically, under-estimated problem.

The theoretical model of paradoxical embolism requires the presence of four parameters, namely, arterial embolism, venous thrombus, abnormal intracardiac communication and right-to-left shunt. Many aspects, however, of this well known entity are under consideration; diagnosis is often difficult to be established and the long term efficacy of preventive measures is undefined. We comment on a case report of recurrent paradoxical embolism with popliteal vein thrombosis and patent foramen ovale, and we briefly review the literature.

Acute right heart failure due to adenotonsillar hypertrophy.

A case is presented in which a child with underlying chronic lung disease, developed cor pulmonale and severe pulmonary hypertension as a result of adenotonsillar hypertrophy. His cardiac function acutely decompensated with an upper respiratory infection which exacerbated his obstructive sleep symptoms. Pre and postoperative documentation of cardiopulmonary function was critical in the peri-operative management of this patient. His severe pulmonary hypertension was stabilized using a nasopharyngeal airway and medications pre-operatively, in order to minimize his risk of anesthesia. He continued to require careful monitoring and manipulation of his medications after adenotonsillectomy and bronchoscopy. Serial echocardiograms documented the effects of the various interventions implemented in this patient. Severe acute right heart failure is an unusual complication of obstructive sleep apnea. However, this may become more common as more children survive prematurity and its associated chronic lung disease. These children may have subclinical lung disease and/or chronic pulmonary hypertension even after they no longer require supplemental oxygen and bronchodilators. Because these children are often tenuous, with regard to their cardiopulmonary function, they may be at increased risk to develop significant complications related to obstructive sleep apnea. It is important that a physician familiar with the management of pulmonary hypertension be involved in the care of these patients.

[Chronic cor pulmonale and refractory hypoxemia following pulmonary embolism in a six-month-old infant: surgical management by thromboendarterectomy]. / Coeur pulmonaire chronique postembolique et hypoxémie réfractaire chez un nourrisson de six mois: prise en charge chirurgicale par thromboendartériectomie.

UNLABELLED: A diagnosis of pulmonary embolism is uncommon in the infant and the child, and chronic cor pulmonale secondary to pulmonary embolism is an even rarer occurrence. CASE REPORT: In this study, a case of pulmonary embolism in a 6-month-old male infant has been reported. His past history included preterm birth, and severe bronchopulmonary dysplasia, with prolonged oxygen dependency. The positive diagnosis was based on cardiac ultrasound examination, with the direct imaging of a right pulmonary arterial thrombus. Surgical thromboendarterectomy was performed, with a long-term favorable outcome. CONCLUSION: After excluding from the diagnosis those hemostatic disorders known to be thrombogenic, the most likely hypothesis was retained, i.e., that it was catheter-related. A central venous catheter had been inserted during the neonatal period, and was probably responsible for the embolism. The clinical characteristics and the diagnostic and therapeutic aspects of chronic postembolic cor pulmonale have been discussed in the light of the present findings.

[Post-embolic chronic pulmonary heart disease: a case report]. / Coeur pulmonaire chronique post-embolique: à propos d'une observation.

Echocardiography and pulmonary scintigraphy demonstrated post-embolic chronic cor pulmonale in a six-year-old woman presenting with dyspnoea. The patient's symptoms deteriorated during subsequent thromboembolic episodes, despite treatment consisting of oral anticoagulants and diuretics. After discussing the pathophysiology and diagnostic methods, the authors emphasize the limits of medical treatment of post-embolic chronic cor pulmonale and the value of two surgical treatments: thromboendarterectomy and lung transplantation. They also recall the precise selection criteria for lung transplantation.

[Pulmonary thromboendarterectomy: an effective surgical treatment for cor pulmonale due to chronic pulmonary emboli]. / Pulmonalistrombo-endarteriëctomie: een effectieve chirurgische behandeling voor patiënten met cor pulmonale door chronische longembolie.

OBJECTIVE: To evaluate the initial experience with pulmonary thromboendarterectomy for cor pulmonale due to chronic pulmonary embolism. METHOD: In the period 1 April 1996 to 31 October 2001, 18 patients with right ventricular failure due to chronic thromboembolic pulmonary hypertension were operated on. Their mean age was 54 (SD: 14) years. Preoperatively, all of the patients were in functional New York Heart Association (NYHA) class III or IV. Pulmonary angiography revealed pulmonary hypertension with an increased pulmonary vascular resistance, as well as typical angiographic signs of unresolved chronic emboli. Pulmonary thromboendarterectomy was performed via median sternotomy, using extracorporeal circulation and intermittent deep hypothermic circulatory arrest. The patients were monitored via the outpatients' department. RESULTS: From a technical viewpoint, the procedure was performed successfully in all of the patients. Initially the pulmonary thromboendarterectomy was performed unilaterally (n = 7), which did not decrease pulmonary artery pressure significantly. The following 11 patients were treated bilaterally; in them thromboendarterectomy required an average of circulatory arrest totalling 64 (SD: 30) min with 161 (SD: 35) min of myocardial ischemia. After bilateral pulmonary thromboendarterectomy, the pulmonary artery pressure decreased from 45 (SD: 13) to 28 (SD: 9) mmHg (p = 0.001). Reperfusion pulmonary oedema, requiring prolonged ventilation, occurred in 3 patients. There was no operative or later mortality. At a mean follow-up of 28 (SD: 19) months, all but one of the patients were in functional NYHA class I or II. Echocardiography revealed reduced right ventricular dimensions and pulmonary artery pressures. One patient, with mainly distally located obstructions, exhibited no substantial improvement. CONCLUSION: The initial experience with pulmonary thromboendarterectomy for patients with cor pulmonale due to chronic pulmonary emboli demonstrated satisfactory mid-term follow-up data.

Comparison of cardiac allograft vasculopathy in heart and heart-lung transplantations: a 15-year retrospective study.

BACKGROUND: Cardiac allograft vasculopathy (CAV) is a major factor limiting long-term survival after heart transplantation (HTx). Specific determinants of CAV and long-term outcome after CAV occurrence have been poorly investigated after heart-lung transplantation (HLTx). METHODS: Between January 1996 and December 2006, 79 patients underwent HLTx (36.3 ± 12.2 years old; 47% men) and 141 patients underwent HTx (49.2 ± 12.3 years old; 77% men) at two different institutions. CAV grading was reviewed in both groups according to the 2010 standardized nomenclature of the International Society for Heart and Lung Transplantation. The mean post-transplant follow-up was 94 (1 to 181) months. RESULTS: Overall 10-year survival rate was 58% after HTx and 43% after HLTx (p = 0.11). The Grade 1 (or higher) CAV-free survival rate was 95% at 4 years and 69% at 10 years after HLTx, and 77% and 39%, respectively, after HTx (p < 0.01). Mean cyclosporine blood levels were similar between the groups at 3, 6, 12, 24 and 36 months. The main causes of mortality beyond 5 years after HTx and HLTx were malignancies and bronchiolitis obliterans, respectively. By multivariate analysis, recipients who developed >3 acute myocardial rejections during the first year post-transplant were exposed to a higher risk of CAV (95% CI 1.065 to 2.33, p = 0.02). Episodes of acute pulmonary rejection and bronchiolitis obliterans were not associated with an increased risk of CAV (p = 0.52 and p = 0.30). CONCLUSION: HLTx recipients appeared protected from CAV compared with HTx patients in this retrospective study. Repeated acute cardiac rejections were independent predictors of CAV. Unlike bronchiolitis obliterans, CAV had a very low impact on long-term survival after HLTx.

Vasodilator Agents in Pediatric Cardiac Surgery with Cardiopulmonary Bypass

Surgical management of congenital heart disease has become regularly performed in tertiary hospitals worldwide. Although routinely needed, cardiopulmonary bypass (CPB) deleteriously affects physiological processes being among the main causes of increased morbidity in cardiac surgery, especially in neonates and children.Recent studies have shown that vasodilator agents’ use during CPB improved peripheral tissue perfusion while maintaining high flow during bypass. Hence, an improvement in perfusion and oxygenation of the skin, musculature and intestinal mucosa has been demonstrated, with favorable result throughout the postoperative period.These agents, by reducing systemic vascular resistance (SVR), lead to greater peripheral blood flow, improvement in peripheral perfusion, decreased base deficit and lactic acidosis and therefore improved respiratory and circulatory status.The present article reviews the main aspects of vasodilator agents most commonly used in pediatric cardiac surgery with CPB, including: chlorpromazine, phentolamine, phenoxybenzamine, sodium nitroprusside, volatile anesthetic agents, calcium channel blockers, angiotensin blockers and inodilators.

Preserving mitochondrial function prevents the proteasomal degradation of GTP cyclohydrolase I.

The development of pulmonary hypertension is a common accompaniment of congenital heart disease (CHD) with increased pulmonary blood flow. Our recent evidence suggests that asymmetric dimethylarginine (ADMA)-induced mitochondrial dysfunction causes endothelial nitric oxide synthase (eNOS) uncoupling secondary to a proteasome-dependent degradation of GTP cyclohydrolase I (GCH1) that results in a decrease in the NOS cofactor tetrahydrobiopterin (BH(4)). Decreases in NO signaling are thought to be an early hallmark of endothelial dysfunction. As l-carnitine plays an important role in maintaining mitochondrial function, in this study we examined the protective mechanisms and the therapeutic potential of l-carnitine on NO signaling in pulmonary arterial endothelial cells and in a lamb model of CHD and increased pulmonary blood flow (Shunt). Acetyl-l-carnitine attenuated the ADMA-mediated proteasomal degradation of GCH1. This preservation was associated with a decrease in the association of GCH1 with Hsp70 and the C-terminus of Hsp70-interacting protein (CHIP) and a decrease in its ubiquitination. This in turn prevented the decrease in BH(4) levels induced by ADMA and preserved NO signaling. Treatment of Shunt lambs with l-carnitine also reduced GCH1/CHIP interactions, attenuated the ubiquitination and degradation of GCH1, and increased BH(4) levels compared to vehicle-treated Shunt lambs. The increases in BH(4) were associated with decreased NOS uncoupling and enhanced NO generation. Thus, we conclude that L-carnitine may have a therapeutic potential in the treatment of pulmonary hypertension in children with CHD with increased pulmonary blood flow.