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T C Martin

Publications and source records attributed to T C Martin.

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Primary surgical closure of large ventricular septal defects in small infants.

Herein, a policy of primary surgical closure of large ventricular septal defects in infants is reviewed. Forty-eight infants met criteria for inclusion in the study, and were divided into two groups based on weight: group 1 infants weighted 4 kg or less (n = 23), and group 2 infants weighed more than 4 kg (n = 25). Both groups had similar variation in ventricular septal defect location (paramembranous versus muscular) and number (single versus multiple), as well as incidence of major associated extracardiac diseases. No early deaths occurred in group 1, compared with 1 infant (4%) in group 2. Major complications occurred similarly in both groups (9% versus 12%). There were two late deaths in group 1 (9%) and none in group 2. No surviving patients have required a second ventricular septal defect operation, and the majority no longer receive anticongestive therapies. These results indicate that primary surgical closure of large ventricular septal defects, even multiple muscular defects, can be performed in very small infants with no difference in mortality or serious complication rates compared with larger infants. Protracted medical efforts to achieve larger size before primary repair and palliative pulmonary artery banding are not necessary.

Body Weight

Complete repair of total anomalous pulmonary venous connection in infancy.

From 1983 to 1990, 20 infants underwent complete repair of isolated total anomalous pulmonary venous connection. Twelve were male; ages ranged from 1 day to 240 days (mean 32 days). The abnormal anatomic connection was supracardiac in nine, cardiac in four, mixed in five, and infradiaphragmatic in two. In seven patients the pulmonary venous drainage was obstructed. Corrective operations were performed 2.4 days after admission; after medical stabilization, seven patients underwent emergency operations, including extracorporeal membrane oxygenation in one. All operations were performed with the use of circulatory arrest (mean 33 minutes) and cardiopulmonary bypass (mean 54 minutes). There was one hospital death, which occurred in a premature infant (1.5 kg) who had supracardiac drainage. No preoperative risk factors correlated with a poor surgical outcome. Pulmonary hypertensive episodes were expectantly managed after the operation with 100% oxygen, sedation, and hyperventilation. Postoperative arrhythmias occurred predominantly in patients with intracardiac drainage. All survivors (mean follow-up of 42 months) are in sinus rhythm, receiving no medications, and are growing and developing normally. Surgical correction of total anomalous pulmonary venous connection in infancy can be performed at low risk with good results after aggressive preoperative stabilization and postoperative management.

Cardiopulmonary Bypass

Combined deficiency of beta-galactosidase and neuraminidase: natural history of the disease in the first 18 years of an American patient with late infantile onset form.

We describe the clinical findings over the first 18 years of a patient with a novel phenotype for galactosialidosis, the storage disease produced by the combined deficiency of beta-galactosidase and neuraminidase. Clinical findings in the first few months included somewhat unusual appearance and hepatosplenomegaly. Dysostosis multiplex was evident by age 2 1/2 years. Mitral and aortic valvular disease appeared over the next few years and cardiac disease has become the most important clinical problem. Foam cells were present in the bone marrow, and vacuolated lymphocytes were present in the peripheral blood smear. The patient had no neurological symptoms, cherry red spots, or intellectual deterioration during the first 18 years. Evidence presented elsewhere indicates that the basic defect in this late infantile form of galactosialidosis (as is thought to be true for the other forms of galactosialidosis) is a reduced amount of the 32 kDa phosphoglycoprotein which associates with beta-galactosidase and alpha-neuraminidase in lysosomes.

Aortic Valve Insufficiency

Evaluation of pulmonary arterial morphology in cyanotic congenital heart disease by magnetic resonance imaging.

Before and after surgical therapy, the anatomy of the pulmonary arteries in cyanotic congenital heart disease is often distorted. Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI) and angiography in 20 patients, ages 3 months to 20 years, with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r = 0.96; right pulmonary artery, r = 0.93; left pulmonary artery, r = 0.96). A similar excellent correlation (kappa = 0.83) was found in the assessment of the presence and severity of proximal pulmonary arterial stenoses. However, stenoses in the peripheral pulmonary arteries visualized with angiography were missed with MRI. MRI and angiography showed complete agreement in determining the patency of 11 surgical shunts. MRI did not demonstrate all of the systemic collateral vessels present with angiography, and the distal connections of collaterals were not detected with MRI. MRI is comparable to angiography in the evaluation of central pulmonary arterial anatomy over a wide range of ages. These findings suggest an important role for noninvasive MRI in the serial evaluation of pulmonary artery morphology in patients with cyanotic congenital heart disease before and after surgical repair.

Adolescent

Intraoperative evaluation of atrioventricular septal defect repair by color flow mapping echocardiography.

Despite improving survival rates after repair of atrioventricular septal defect, many patients require reoperation because of postoperative heart failure. We used intraoperative color flow mapping echocardiography to assess the results of surgical repair of atrioventricular septal defect in 19 consecutive patients and compared those findings with results three to five days and 3 to 11 months after repair. There was close correlation between intraoperative and postoperative color flow mapping echocardiography in estimating the presence and severity of left atrioventricular valve regurgitation. All patients survived surgical repair, but in 4 (21%), postoperative congestive heart failure due to left atrioventricular valve regurgitation developed. The need for reoperation was significantly correlated with the severity of left atrioventricular valve regurgitation (r = 0.68) as estimated by intraoperative echocardiography and preoperative aberrancies in the atrioventricular valve (r = 0.68). Age, weight, additional congenital heart disease, preoperative pulmonary vascular resistance, preoperative atrioventricular valve regurgitation, and postoperative mean pulmonary arterial or left atrial pressure were not significantly correlated with the need for reoperation. Intraoperative color flow mapping echocardiography can accurately predict the development of early postoperative heart failure and subsequent reoperation after surgical repair of atrioventricular septal defect.

Child

Management and follow-up of arterial thrombosis in the neonatal period.

The management and follow-up of 12 patients with major aortic thrombus formation occurring in the neonatal period between 1982 and 1987 are reported. Umbilical arterial catheters were inserted in 8 of the 12 patients before thrombus formation. Two patients had congenital thrombi. Hypertension, oliguria, hematuria, and elevated blood creatinine concentration were found at the time of diagnosis of the thrombus; nine of the patients had a patent ductus arteriosus. Supportive care was instituted in seven patients who were hemodynamically stable. Five of the patients had congestive heart failure, shock, or both, and were treated with surgical thrombectomy. Thrombolytic therapy was not used in either group. The five surgically treated patients and six of seven medically treated patients survived. Ultrasound examination suggested resolution of the thrombus in all survivors in 6 to 30 days. Sequelae from thrombus formation were present in all patients at the time of discharge and included hypertension in 9 of the 11 survivors and decreased renal function in six of them. Follow-up at 1 to 3 years revealed normal blood pressure, good growth, and good renal function in 10 of the survivors.

Anticoagulants

Recent trends in marital disruption.

The post-1980 decline in the crude divorce rate must be interpreted in the context of the long-term trend and in terms of what we know about composition effects on crude measures-particularly given shifts in age at marriage and the age composition effects of the baby boom. Data from the June 1985 Current Population Survey permit more detailed, exposure-specific measurements as well as the use of separation as the event terminating marriage. Estimates from these data suggest a decline followed by a recovery. Taking into account well-known levels of underreporting, we find that recent rates imply that about two-thirds of all first marriages are likely to end in separation or divorce. We examine the persistence of major differences in marital stability and evaluate the comparative stability of first and second marriages.

Age Factors

Dilated cardiomyopathy in infants and children.

The outcome of medical treatment of dilated cardiomyopathy in infants and children was reviewed to develop a predictive index for selection of patients likely to benefit from cardiac transplantation. The clinical findings, laboratory investigations, treatment and outcome of 20 patients (Group 1) less than 2 years of age at presentation and 12 patients (Group 2) greater than 2 years of age at onset were compared. Of 20 Group 1 patients, 5 (25%) died. Available autopsies (four patients) showed endocardial fibroelastosis. Of 15 survivors, 10 showed improvement in cardiac status and 5 remained unchanged. Ninety-three percent of survivors had dilated cardiomyopathy consistent with endocardial fibroelastosis by angiocardiography. All 12 Group 2 patients died. In addition to age at presentation and poor outcome, Group 2 differed from Group 1 in having a higher incidence of other family members with cardiomyopathy, more significant rhythm disturbances at presentation and a more rapid course to death. Risk factors of poor outcome in both groups included persistent cardiomegaly and the development of significant arrhythmias by Holter electrocardiographic monitoring. Cardiac transplantation is recommended for children with dilated cardiomyopathy presenting after age 2 years who survive 1 month. Those patients less than 2 years old at presentation whose condition has not improved after 1 year and who have persistent cardiomegaly or complex ventricular arrhythmias may also benefit from transplantation.

Actuarial Analysis

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Anesthetics, Local