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Query: UMLS:C0042373 (vascular disease)
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Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.
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PMID:Repair of truncus arteriosus in the neonate and young infant. 271 23

Pulmonary vascular structure was analyzed in lung tissue taken from 38 patients, aged 2 weeks to 9.1 years, with complete atrioventricular (AV) septal defect without important left AV valve regurgitation. Biopsy material was used in 27 patients (71%). Pulmonary artery (PA) pressure and resistance increased with age (r = 0.5, p less than 0.001 and r = 0.4, p less than 0.02, respectively). Intra-acinar mean PA arterial medial thickness decreased with age (r = -0.6, p less than 0.001), PA pressure (r = -0.6, p less than 0.001) and resistance (r = -0.4, p less than 0.05). Reduction in intra-acinar PA muscularity was associated with an increase in severity of obstructive intimal damage in pre-acinar PAs with age. A PA resistance of less than 6 units m2 was associated with age younger than 3 years (usually younger than 2 years), PA medial thickness of more than 19% (normal = 7.4%), and muscle extension with or without intimal proliferation. A higher resistance occurred in patients of all ages, but in those younger than 3 years structural abnormalities usually resembled those in patients with a lower resistance, whereas older patients had a normal or slightly increased PA medial thickness of 14% or less (normal = 7.4%), or classic grade III or IV disease. The 4 perioperative deaths attributed to pulmonary vascular disease occurred in patients who were similar in age and hemodynamic status to the survivors, but who had more severe pulmonary vascular abnormalities; either less muscularity with more severe intimal fibrosis or a much greater increase in muscularity, emphasizing that even potentially reversible abnormalities can prejudice intracardiac repair.
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PMID:Pulmonary vascular bed in children with complete atrioventricular septal defect: relation between structural and hemodynamic abnormalities. 293 64

We have developed real-time two-dimensional Doppler echocardiography, which can display color flow mapping on a two-dimensional echocardiogram. Intraoperative two-dimensional Doppler echocardiography was performed during cardiovascular operations before and after the definitive procedure in 33 patients, 14 with valvular disease, eight with congenital heart disease, and 11 with vascular disease. Its clinical usefulness was evaluated. In patients with valvular heart disease, 13 valve replacements, 10 valvuloplasties, and four untouched lesions were assessed. No paravalvular leakage and three instances of minimal physiological transvalvular leakage from St. Jude Medical valves in the mitral position were noticed. Regurgitation after valvuloplasty was graded by extent of the regurgitant flow. This grading, comparable to postoperative grading, indicated no need for an additional procedure. In congenital heart disease, preoperative diagnoses were confirmed. The effect of the corrective operation was evaluated and no significant leakage or stenosis was found. Interatrial shunt flow was shown to increase after operative balloon atrial septostomy in a patient with dextro-transposition of the great arteries. In 11 patients with dissecting aortic aneurysm, the aneurysm was totally visualized in the operative field, including the structure and flow dynamics. In two patients, the preoperative cineangiographic diagnosis regarding involvement of dissection was corrected. After the vascular procedure, sufficient flow in the major aortic branches was confirmed in all patients and minimal leakage at the suture line was noticed in four patients. In conclusion, intraoperative color flow mapping by two-dimensional Doppler echocardiography has enabled the precise diagnosis and the necessary operation to be determined before cardiopulmonary bypass. It has also allowed the effects of the operation to be assessed before chest closure.
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PMID:Intraoperative color flow mapping by real-time two-dimensional Doppler echocardiography for evaluation of valvular and congenital heart disease and vascular disease. 406 30

Six cases of this rare association (7 to 10 p. 100 of Takayasu's disease) are reported. The authors also review 73 previously reported cases. The valvular lesion is usually detected secondarily during follow-up (2/3 of cases), but is observed at the same time as the arterial disease in about 1 out of 5 cases. In rare cases, it may be the presenting feature before the vascular disease becomes clinically apparent. Quantification of the regurgitation may be difficult because of stenosis of the thoracic aorta and the supra aortic vessels. In some cases it is severe and poorly tolerated but has no particular distinguishing features apart from the incidence of aortic wall calcification (ascending aorta to all of the aortic arch). Twenty five anatomical observations (operative or autopsy) are sufficiently well documented to show the mechanism of the aortic incompetence. It is caused by an inflammatory aortitis: valvular lesions were found in 2/3 of cases but other causes may be observed, dilatation of the aortic ring (1 out of 4 cases), disunion of the commissures (1 out of 4 cases) and changes in aortic compliance causing systolic hypertension. Aortic valve replacement is rare (11 cases including 3 personal cases) and sometimes completed by resection of an aneurysmal ascending aorta.
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PMID:[Aortic insufficiency and Takayasu disease]. 614 55

Fourteen patients younger than two years of age with persistent truncus arteriosus underwent primary repair. Twelve of them were less than 1 year and 4 less than 3 months of age. Intractable heart failure was the indication for surgery in all patients but one who had increased pulmonary vascular resistance. There were 5 hospital and 2 late deaths. Six out of the 7 survivors (median follow-up: 29 months) were symptom-free. The remaining infant who preoperatively had significant truncal valve regurgitation was doing fairly well 2 1/2 years after repair. Our experience suggests that, although the mortality remains high, primary repair for infants with persistent truncus arteriosus is feasible and offers better overall results than does pulmonary artery banding followed by later intracardiac repair. We advise primary repair for all infants with intractable heart failure or increasing pulmonary vascular resistance with or without truncal valve regurgitation. Elective repair is recommended before the age of 2 years to minimize the risk of pulmonary vascular disease.
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PMID:Surgical repair of persistent truncus arteriosus in infancy. 615 30

Between 1971 and 1980, 65 children, aged 2 weeks to 15 years (mean 6.8 years) had "fresh" antibiotic sterilized aortic homografts inserted as a valved external conduit. Thirty-six patients (55%) had undergone previous palliations. Operations were performed on cardiopulmonary bypass, with hypothermia and cardioplegia. In selected young infants, deep hypothermia with circulatory arrest was used. Twenty-five patients (38%) died after the operation. Mortality was related to the complexity of the lesion, the condition of the child on admission, and the degree of pulmonary vascular disease. In addition, there were 7 late deaths. Twenty-one patients were recatheterized, either as a part of routine postoperative assessment (13) or because of symptoms (8). Satisfactory conduit performance, judged by the absence of significant gradients or regurgitation, was found in 18 out of 21 restudied patients. Calcification of the homograft aortic wall was seen on chest X-ray in 56% of patients. The aortic valve calcified in only one child, following an episode of subacute bacterial endocarditis. We conclude that fresh antibiotic preserved aortic homografts perform well in extracardiac valved conduits. They are easy to insert and better hemostasis can be achieved. Degeneration of the valved leaflets is extremely rare.
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PMID:"Fresh", antibiotic sterilized aortic homografts in extracardiac valved conduits. Long-term results. 619 66

To elucidate the factors which are associated with early and late operative results of atrioventricular septal defects, 102 consecutive patients who underwent reparative operation of atrioventricular septal defects (AVSD) in our institution since 1968 were studied. Our operative technique was basically the same through this period. That is the two patch method in its complete form (the so-called Shirotani's method) and preferential use of cleft closure supplemented with Kay-Reed-Wooler type annuloplasty. Early mortality (< 30 days) rate was 18.6%. More than 80% of the early deaths were not related to atrioventricular valve malfunction. Non-complete closure of the cleft, high preoperative pulmonary vascular resistance, deficient atrioventricular valve, and complete form showed independent correlations with early mortality in multiple logistic regression analysis. For operative survivors, event-free survival curves, for atrioventricular valve related reoperations were drawn for various factors. Two late deaths and 3 late atrioventricular-valve-related reoperations occurred. The event-free actuarial survival for operative survivors at 5, 10, and 20 years were 97.0%, 89.4%, and 89.4%, respectively. The survival analysis revealed that preoperative high pulmonary vascular resistance, preoperative severe atrioventricular regurgitation, and preoperative large cardiothoracic ratio in chest radiogram were related with late event occurrence. Higher early mortality in our series may be attributable to relatively advanced pulmonary vascular occlusive disease rather than post repair atrioventricular valve malfunction. On the other hand, our late results were rather good. We concluded that the Shirotani's method and preferential use of cleft closure supplemented with Kay-Reed-Wooler type annuloplasty was effective for most of atrioventricular valves in atrioventricular septal defects. Early surgical intervention before pulmonary vascular disease progression or atrioventricular valve regurgitation development is also important.
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PMID:Long term follow-up of atrioventricular valve function after repair of atrioventricular septal defect. 1033 13

Nowadays, it is estimated that 85% of the infants born with congenital heart disease (CHD) will survive to adulthood, thanks mainly to surgical or therapeutic procedures performed during infancy or childhood. The clinical profile and disease pattern of adults with CHD is changing. The prevalence of certain adult CHDs, such as tetralogy of Fallot, transposition of the great arteries or univentricular heart, is rising, but these conditions have practically become new diseases as a result of therapy. Most surviving patients present residua, sequelae, or complications, which can progress during adult life. These disorders can present electrophysiological disturbances, valvular disease, persistent shunts, myocardial dysfunction, pulmonary or systemic vascular disease, problems caused by prosthetic materials, infectious complications, thromboembolic events, or extravascular disorders involving multiple organs or systems. In tetralogy of Fallot, the most striking problems that affect long-term prognosis are pulmonary valve regurgitation, right ventricle dysfunction, and atrial or ventricular arrhythmias. The main problems appearing after physiological atrial repair of transposition of the great arteries are related to right ventricular function, since it is structurally unprepared for systemic circulation, and atrial arrhythmias. Surgical repair of univentricular heart using Fontan techniques should be considered a palliative procedure that does not modify the underlying structural disorder and exposes the postoperative patient to severe complications and problems. The increase in the number of patients with CHD who will reach adulthood in the coming decades makes it necessary to carefully consider the new healthcare demands that are being generated, who should be responsible for them, and how and where solutions can be found.
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PMID:[Congenital heart disease in adults: residua, sequelae, and complications of cardiac defects repaired at an early age]. 1255 3

Preoperative pulmonary vascular disease remains an important risk factor for death or right-heart failure in selected children undergoing two-ventricle repair, single-ventricle palliation, or heart transplantation. Preoperative criteria for poor outcome after operation remain unclear. The purpose of this review is to critically assess both the historic and current data and make recommendations where appropriate. An extensive literature search was undertaken in October 2009. Data were analyzed by an expert multidisciplinary team and recommendations were made by consensus. PubMed was searched in October 2009. Data were analyzed and recommendations were made by consensus of a multidisciplinary team. In patients with suspected pulmonary vascular disease anticipating a two-ventricle repair, although preoperative testing via cardiac catheterization with vasodilators is reasonable, the preoperative parameters and the precise values of these parameters that best correlate with early and late outcome remain unclear. Further investigation is warranted in selected populations, such as the growing group of children with congenital heart disease complicated by chronic lung disease of prematurity, and in the developing world where patients may be more likely to present late with advanced pulmonary vascular disease. In patients with a functional single ventricle, there is growing evidence that mean pulmonary artery pressure of >15 mm Hg may be associated with both early and late mortality after the Fontan operation. The relationship of preoperative pulmonary hemodynamics to early and late morbidity remains to be defined. There most likely is a level of preoperative pulmonary vascular disease that puts an individual patient at increased risk for death or severe cyanosis after a bidirectional cavopulmonary anastomosis. It remains unclear, however, how to best assess this risk preoperatively. The limitations in obtaining an accurate assessment of pulmonary vascular disease in the complex single ventricle are discussed. In children awaiting cardiac transplantation with elevated pulmonary vascular disease of >6 U.m and/or transpulmonary gradient of >15 mm Hg, heart transplantation is deemed feasible in most transplant centers if the administration of inotropes or vasodilators can decrease the pulmonary vascular disease to <6 U.m or transpulmonary gradient to <15 mm Hg. In patients with preoperative pulmonary vascular disease, there may be contributing factors to the pulmonary vascular disease, such as the specifics of the cardiac lesion (atrioventricular valve regurgitation, low cardiac output), parenchymal and/or airway issues, and/or individual genetic predisposition. Amelioration of any reversible factors before operation and optimization of their management in the preoperative and postoperative period are recommended.
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PMID:Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation? 2021 66