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Biomedical subjects

E Arciniegas

Publications and source records attributed to E Arciniegas.

At least 37 records · Page 2Linked to original sources

Primary pulmonary artery sarcoma in two children.

The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.

Adolescent↗

Growth response of endothelial and smooth muscle cells to a collagen matrix throughout development.

The in vitro response of chick embryo endothelial and smooth muscle cells from aorta explants, induced to grow on a collagen type I matrix, was investigated at different stages of development. At early stages of embryonic development both cell types reacted with collagen as a substratum. At later stages, the endothelial cells had a lower response, whereas the response of muscle cells remained constant. Further evidence is given for the spatial-specific behaviour demonstrated by endothelial cells, which remained on the surface while smooth muscle cells invaded the matrix. Results further highlight the possible role of endothelial cell polarity on the cell-collagen interaction and growing response.

Animals↗

Surgical and medical results of complete atrioventricular canal: a ten year review.

The files of 121 patients who presented to Children's Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Cardiac Catheterization↗

Pulmonary hypertension postventricular septal defect repair treated by extracorporeal membrane oxygenation.

Severe pulmonary hypertension complicating the correction of congenital cardiac defects is an unusual cause of early postoperative mortality. We present a case of a nine-month-old infant who developed paroxysmal pulmonary hypertension associated with severe hypoxemia after the successful repair of a large perimembranous ventricular septal defect (VSD). The pulmonary hypertension was refractory to all medical and pharmacologic therapy but was successfully treated with extracorporeal membrane oxygenation (ECMO). On ECMO, pharmacologic support was removed, pulmonary artery pressure reduced, and ECMO support withdrawn. To date, ECMO has been applied to pulmonary hypertension of the newborn, neonatal respiratory insufficiency, and for primary cardiac pump failure. Our experience with this case leads us to believe it is an effective therapy for acute pulmonary hypertension occurring after the repair of congenital cardiac anomalies.

Heart Arrest↗

Asymptomatic intracardiac metastasis from osteosarcoma: a case report with literature review.

Osteosarcoma very rarely metastasizes to the heart. Thirteen cases have been reported in the literature so far. Diagnosis in some of these cases was made during investigation for severe cardiac failure and in most of them at autopsy. Our patient, a 13-year-old girl, showed right pulmonary metastases on chest x-ray 1 year after above knee amputation for osteosarcoma of the distal femur. Routine preoperative computerized axial tomography (CT Scan) revealed a calcified lesion in the heart in addition to the pulmonary metastases. She was very active and completely asymptomatic. Two-dimensional echocardiography, angiography, and right and left heart catheterizations were done. This revealed a large mobile metastatic lesion in the right ventricle. The intraventricular tumor was successfully removed, and 12 days later she had a second thoracotomy for removal of pulmonary metastases. Nine months after her intraventricular metastasis was removed she developed a solitary right pulmonary metastasis. This was successfully resected. Now, 10 months later, she is disease free and completely asymptomatic.

Adolescent↗

Classic shunting operations for congenital cyanotic heart defects.

A total of 297 consecutive classic systemic artery-pulmonary artery shunts were performed in 281 patients over the past 9 years. This experience includes 200 Blalock-Taussig shunts (67.3%), 84 Waterston shunts (28.2%), and 13 Potts shunts (4.3%). The overall early mortality after the Blalock-Taussig shunt (5.5%) was significantly lower (p less than 0.02) than for the Waterston shunt (13%). The early mortality after either the Blalock-Taussig or Waterston shunt was not statistically related to the age of the patient at operation. Both the Blalock-Taussig and Waterston shunts demonstrated a higher mortality (p less than 0.02) among patients in Group II (complex defects) than in patients in Group I (tetralogy of Fallot and pulmonary atresia with ventricular septal defect [VSD]). However, the Blalock-Taussig shunt had a significantly lower (p less than 0.04) probability of early postoperative death than the Waterston shunt among patients with complex lesions. The Blalock-Taussig shunt also revealed a lower incidence of important late postoperative complications and did not affect adversely the hemodynamic result after intracardiac correction, as evidenced by late postoperative cardiac catheterization. This study also confirms the feasibility of the Blalock-Taussig shunt during the neonatal period without significantly increased age-related risk of postoperative death (p = 0.13) and with good early and late long-term patency rates.

Cardiac Output, Low↗

Surgical treatment of cor triatriatum.

Six patients with cor triatriatum underwent surgical correction. They ranged for 1.5 to 93 months old (mean, 22 months). Congestive heart failure was present in 3 patients. Cardiomegaly and increased pulmonary vascularity were evident roentgenographically in all patients. Cardiac cineangiography demonstrated the subdividing left atrial membrane in 5 patients and suggested the correct diagnosis by revealing an abnormal configuration of the left atrium in the other patient. The opening in the anomalous left atrial membrane was stenotic in every instance. The proximal left atrial chamber communicated with the right atrium through an atrial septal defect in 5 patients and with the systemic venous circuit through a persistent left superior vena cava in the other patient, in whom the atrial septum was intact. A right atrial-transseptal approach provided ample exposure for complete excision of the obstructing membrane and repair of the atrial septum in all patients. One patient died of low cardiac output during the early postoperative period. The other 5 are alive and well at an average of 48 months after operation.

Blood Pressure↗

Results of the Mustard operation for dextro-transposition of the great arteries.

One hundred twenty patients with dextro-transposition of the great arteries (TGA) underwent intra-arterial baffle repair using thin Dacron fabric from 1971 to 1979. The ages of the patients ranging from 29 days to 17 years (mean age 28 months). Thirty-five patients had undergone 49 preliminary palliative operations. Early postoperative mortality was 4.8% for patients with simple TGA but was higher among patients with associated ventricular septal defect (26%), VSD and left ventricular outflow tract obstruction (28.5%) and intact ventricular septum with left ventricular outflow tract obstruction (12.5%). Early nonfatal postoperative complications included low cardiac output (23.3%), respiratory insufficiency (35.8%), junctional rhythm (34.1%), superior vena caval (SVC) obstruction (9.1%), and chylothorax (7.5%). Late postoperative mortality for all groups was 7.5% (nine patients). Late cardiac dysrhythmias occurred in 33 patients (40.7%). Normal sinus rhythm has been preserved in all patients since direct, high SVC cannulation was instituted. Late postoperative hemodynamic and angiographic evaluation in 61 patients revealed severe to total SVC obstruction in 12 patients (20.2%) and pulmonary venous obstruction in four patients (6.5%). Among the latter, two patients died following reoperation and another patient died without operation. SVC obstruction was clinically important in only three patients, two of whom have undergone successful reoperation. All other late survivors are acyanotic and clinically well. The Mustard operation has dramatically improved the survival rate and quality of life for patients with TGA. However, postoperative caval and pulmonary venous obstruction are problems which require additional technical modifications and stimulate the search for alternative corrective operations.

Adolescent↗

Results of total correction of tetralogy of Fallot with complete atrioventricular canal.

Four patients with tetralogy of Fallot (TF) and complete atrioventricular canal (CAVC) underwent successful intracardiac repair. All patients had undergone preliminary palliative shunts. Associated cardiovascular lesions included muscular ventricular septal defect (VSD) and left superior vena cava (one patient), bilateral main pulmonary artery stenosis (one patient), and right aortic arch (two patients). The diagnosis was suspected clinically by electrocardiographic evidence of right ventricular hypertrophy and left anterior hemiblock, by echocardiographic findings suggestive of CAVC with aortic dextroposition, and by the association with trisomy 21. The diagnosis was confirmed by cardiac catheterization and cineangiography. Total correction consisted of closure of the VSD and atrial septal defect (ASD), reconstruction of the atrioventricular valves, and relief of the right ventricular outflow tract obstruction (RVOTO). There were no early or late postoperative deaths. Surgically induced complete heart block did not occur. One patient underwent successful reoperation for residual VSD and right ventricular outflow aneurysm. Late hemodynamic evaluation revealed good results in all patients. We recommend a staged treatment plan consisting of preliminary systemic--pulmonary artery shunting for symptomatic children under 4 to 5 years of age and total correction for older children. With proper preoperative diagnosis and accurate intracardiac repair, good results may be obtained after total correction of TF and CAVC.

Adolescent↗

Stenosis of the coronary sinus ostium. An unusual site of obstruction in total anomalous pulmonary venous drainage.

A patient with total anomalous pulmonary venous connection to the coronary sinus and stenosis of the right atrial opening of the coronary sinus is described. While the initial studies were compatible with increased pulmonary blood flow, subsequent evaluation demonstrated severe pulmonary venous obstruction. Successful operative correction was performed.

Coronary Vessel Anomalies↗

Intrapericardial teratoma in infancy.

Intrapericardial teratoma is a rare, congenital, pedunculated tumor, usually attached to the ascending aorta and associated with massive pericardial effusion, cardiac compression, and severe cardiorespiratory distress in infants and young children. Echocardiography and cardiac cineangiography are diagnostic and should be used promptly. The tumor is generally benign and early surgical removal is curative. The case reports of two infants who underwent successful surgical removal of their lesions are presented.

Female↗

Primary cardiac tumors in children.

Case histories of five children with primary cardiac tumors are presented. The clinical and pathological features of the most frequent cardiac neoplasms in childhood are also reviewed. Cardiac tumors should be suspected in any child with unexplained heart murmurs, congestive heart failure, or cardiac arrhythmias. Echocardiography, cardiac catheterization, and biplane cineangiograms provide diagnostic confirmation. Prompt surgical resection is indicated in all symptomatic patients and in those with significant inflow and outflow tumor-related obstruction. Complete resection is not possible in many patients with cardiac rhabdomyoma or lipoma but removal of the obstructive portion of the tumor frequently provides long-term symptomatic relief.

Child↗

Results of two-stage surgical treatment of tetralogy of Fallot.

One hundred nine consecutive patients underent 115 palliative shunts between 1971 and 1979 for the initial management of symptomatic tetralogy of Fallot. A single Blalock-Taussig shunt was done in 78 patients, including 38 infants below 12 months of age, with two deaths (2.5% mortality rate). Thirty-two patients underwent a Waterston shunt, with one death (3.1% mortality rate). The total early shunt mortality rate was 2.7% (three deaths among 109 patients). There was one later noncardiac death and one instance of nonfatal brain abscess between the two stages. Fifty-nine of the previously shunted patients have undergone total correction at a mean postshunt interval of 37.1 months, with one early postoperative death (1.6% mortality rate). The mean patient age at total repair was 4.8 years. The two-stage combined operative mortality rate was 3.6% (four deaths among 109 patients). The Waterston shunt is considered undesirable because it complicated the total repair and was associated with a high incidence of residual right pulmonary artery obstruction at postoperative cardiac catheterization. The Blalock-Taussig shunt had a low mortality rate, high long-term patency rate, absence of interval complications, and no adverse effect at the time of total correction. It is considered the shunt of choice in all symptomatic infants and small children with tetralogy of Fallot. Our experience suggests that, at the present time, the two-stage surgical approach compares favorably with primary total correction, especially in infants under 1 year of age.

Age Factors↗

Early and late results of total correction of tetralogy of Fallot.

Two hundred and nine patients underwent total repair of tetralogy of Fallot without congenital pulmonary atresia from 1971 to 1979. The age of the patients ranged from 22 months to 27 years (mean 6.8 years). Previous palliative shunts were present in 130 patients (62.2%). The right ventricular outflow tract obstruction was relieved by infundibulectomy in 31 patients (14.8%), by transannular patching in 142 patients (67.9%), and by insertion of a right ventricular--to--pulmonary artery prosthetic conduit in 10 patients (4.7%) with associated coronary artery anomalies. A porcine valve was inserted after transannular patching only in patients with undersized pulmonary arteries, unilateral pulmonary artery, or congenitally absent pulmonary valve. There were 10 early postoperative deaths (4.7%). Previous palliative shunts did not adversely affect early postoperative mortality rate. Surgically induced permanent complete heart block occurred in one patient (0.4%). Six patients (3%) died in the late postoperative period, three of them of unrelated causes. Late results were good in 170 patients (87%), fair in 10 patients (5.1%), and poor in 15 patients (7.6%). Poor results were due to residual stenosis at the origin of the pulmonary arteries in 13 patients (6.5%) and to residual ventricular septal defect in two patients (1%). The mean cardiothoracic ratio was 0.61 for patients with poor results and 0.54 for the entire group. Pulmonary valvular insufficiency was well tolerated postoperatively in the absence of distal pulmonary artery obstruction. This experience supports a policy of aggressive relief of the right ventricular outflow tract obstruction including liberal use of transannular patching and, when indicated, extensive reconstruction of the pulmonary artery branches. We also recommend a two-stage treatment program for symptomatic infants with unfavorable anatomy consisting of initial Blalock-Taussig shunt followed by total repair at about 3 years of age.

Adolescent↗