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Biomedical subjects

C E Mullins

Publications and source records attributed to C E Mullins.

At least 109 records · Page 6Linked to original sources

Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transvenous balloon dilation.

Congenital stenosis of the pulmonary veins is a rare but frequently lethal congenital cardiac abnormality. Eight patients with this malformation were diagnosed, evaluated and treated. All eight patients had associated congenital cardiac defects. Two of the eight died, one of sepsis and one after operative pulmonary venoplasty. In three patients who underwent transvenous balloon catheter dilation of the stenosis the procedure provided immediate but transient relief of the stenosis. The prognosis for symptomatic infants with pulmonary vein stenosis is poor and its treatment an enigma.

Angioplasty, Balloon↗

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patient's ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.

Arteries↗

Pulmonary venous dilatation in pulmonary veno-occlusive disease.

A case of pulmonary veno-occlusive disease in an adult is presented. The diagnosis was suspected clinically and verified at cardiac catheterization. Balloon dilatation of the pulmonary veins was attempted at operation and later again in the catheterization laboratory. The result was a significant reduction in pulmonary venous to left atrial pressure gradient and angiographic evidence of increase in venous diameter. It appears that in the focal form of pulmonary venous occlusion, balloon dilatation may offer a therapeutic approach in an otherwise progressively lethal disease.

Adult↗

Congenital coronary artery obstruction associated with aortic anomalies in children: report of two cases.

Coronary arterial obstruction associated with congenital aortic valve disease is rare in children. We studied two children with aortic valve disease and symptoms of coronary insufficiency. Cineangiography revealed localized obstruction of the proximal left coronary artery. In one patient, a membrane-like structure partially covered the left sinus of Valsalva, almost isolating the ostium of the left coronary artery; the other patient had severe stenosis of the proximal left coronary artery and a congenitally hypoplastic left coronary cusp. Surgical repair was successful in both cases. Symptoms of myocardial ischemia in children with aortic valve disease should be investigated promptly to exclude obstruction of the coronary arteries.

Aortic Valve↗

Ventricular septal defect with associated aortic valve insufficiency. Progression of insufficiency and operative results in young children.

Thirty-two children with both ventricular septal defect (VSD) and aortic insufficiency (AI) were evaluated for progression in degree of AI as well as effectiveness of operation in reducing or eliminating regurgitation. At the time of initial study. AI was mild in 21, moderate in eight, and severe in three patients. Twenty-one patients were followed medically over a 2 year to 19 year period, during which the degree of AI progressed in five. One death occurred during an episode of endocarditis. A total of 24 patients underwent operation at ages ranging from 1 year to 23 years. Postoperative evaluations continued from a minimum of 1 year to 24 years in 19 patients, with residual AI noted in 63% of these. Children less than 5 years of age, even with little valvular involvement, exhibited minimal benefit from attempted correction compared with those in the latter part of the first decade of life (p less than 0.05). Closure of the VSD with associated valvuloplasty resulted in a higher prevalence of elimination or improvement of AI compared with VSD closure alone (p less than 0.01). As AI can progress slowly without compromising the child's development, attempted surgical repair can safely be deferred in children less than 5 years of age with minimal valvular involvement unless careful medical management fails to prevent cardiac decompensation.

Adolescent↗

Percutaneous sheath brachial vein cardiac catheterization in children.

During the past ten years we have performed certain phases of cardiac catheterization on 48 children using a modified percutaneous sheath technique to enter a superficial arm vein. Our patients ranged in age from 15 months to 21 years, with a mean age of 10.0 years. The indications for a superior approach most commonly have included electrophysiologic investigations, femoral vein occlusions, and proximal obstructions of the inferior vena cava. The advantages of a percutaneous sheath technique in the superficial brachial veins include the ease of changing catheters frequently during a case and the preservation of the vessel for possible repeat studies. It is not always possible to enter the heart with this technique, owing to sharp angulation of deep venous confluences in the axilla and occasional venous spasm. When successful, this mode of approach is attended by a very low prevalence of complications. There were no deaths, heart or vein perforations, or systemic infections. One patient developed a superficial phlebitis; this was mild and temporally limited. We have not encountered any case of clinically apparent venous thrombosis. When a superior approach for prograde heart catheterization in children is indicated, we have found that our modified percutaneous sheath technique is a simple, safe, and frequently successful alternative to a cutdown approach.

Adolescent↗

Pulmonary artery sling.

Pulmonary artery "sling" indicates the vascular anomaly wherein the left pulmonary artery arises from the right pulmonary artery and traverses between the esophagus and trachea to enter the left lung. Respiratory symptoms may result from compression of the trachea and right mainstem bronchus by the anomalous vessel as well as by associated tracheobronchial defects. Six cases are described. The distinctive radiologic features include anterior indentation of the barium esophagogram and a characteristic pulmonary angiogram. Surgery is the usual recommended treatment for life-threatening airway obstruction, but results are often poor. In patients with less severe symptoms the physician's approach may be modified by the presence of associated intracardiac defects and intrinsic airway disease.

Airway Obstruction↗

Hemodynamic effects of dobutamine in children.

Dobutamine is useful for augmenting cardiovasuclar function in adults. However, no information is available on the action of dobutamine in children. To determine its hemodynamic effects in children, we infused dobutamine into 12 children with congenital heart disease during diagnostic cardiac catheterization. We administered dobutamine in two doses: first 2 and then 7.75 microgram/kg per min for 10 minutes each. We meaured heart rate, cardiac output, systemic and pulmonary arterial, right atrial and pulmonary capillary blood pressures before and during the infusion of dobutamine. Systemic and pulmonary vascular resistances, cardiac index and stroke index were calculated. Cardiac output, cardiac index, stroke volume, stroke index and systemic arterial phasic and mean blood pressures increased sugnificantly (P less than 0.05) and pulmonary capillary mean blood pressure decreased significantly (P less than 0.05) during the infusion of each dose of dobutamine compared with control values. Heart rate, pulmonary and right atrial mean blood pressure and systemic and pulmonary vascular resistance were unchanged with either dose of dobutamine. We noted no adverse effect from the drug.

Adolescent↗

Electrophysiologic studies in patients with ventricular inversion and "corrected transposition".

We evaluated the intracardiac conduction intervals using His bundle recordings in 40 patients with ventricular inversion and 1-transposition of the great arteries. Twenty-nine subjects had 1:1 atrioventricular (AV) conduction. In 15 of those with normal PR intervals and QRS durations, the conduction intervals were not different from those of subjects with normal hearts. In the 14 patients with first-degree AV block, the block was located between the sinus node and AV node in four, between the low right atrium and bundle of His in seven, and below the common bundle of His in four. In 11 subjects with complete AV block, the stie of block was above the site of the His potential in four, below in two and within the His bundle in one. In four patients we could not record a His potential and thus could not localize the site of block. Complete block below the His recording site was associated with syncope in one patient and sudden death in another. His bundle recording is a safe technique for studying the conduction system in children with ventricular inversion and 1-transposition of the great arteries.

Adolescent↗

Cardiac catheterization in the neonate. A comparison of three techniques.

This study evaluated three methods of catheter entry for cardiac catheterization in neonates less than one week of age. Eighty-five neonates underwent venous catheterization through the umbilical vein, 77 by saphenous or femoral cutdown, and 31 by femoral percutaneous entry. Of these 194 neonates, 128 also had an aortic catheter introduced via the umbilical artery. Umbilical vein catheterizations took an average of 115 minutes, 30 minutes shorter than either of the other techniques. Fatal complications following catheterization occurred in 10 of the 194 neonates (5%) three of these were related to catheterization technique. Diagnostic cardiac catheterization and balloon atrial septostomy may be performed most quickly by way of the umbilical vessels. Percutaneous entry of the femoral vein in this series carried the lowest complication rate; cutdown entry resulted in the highest complication rate.

Cardiac Catheterization↗

The absent pulmonary valve syndrome. Considerations of management.

The absent pulmonary valve syndrome consists of a severely hypoplastic pulmonary valve with anular stenosis, aneurysmal dilatation of the main pulmonary artery with one or both pulmonary branches also dilated, and a ventricular septal defect. From 1955 to 1975, 15 patients were seen at Texas Children's Hospital with this syndrome. Although anatomically similar to tetralogy of Fallot, the most significant symptoms during early infancy were secondary to bronchial compression resulting from the dilated pulmonary arteries and enlarged left atrium. Intracardiac repair performed on two infants at four months of age failed to ameliorate the respiratory symptoms and both died. Seven patients from 2.3 to 16 years of age were operated upon, with one late postoperative death. Five other patients have not yet received surgical intervention. We recommend vigorous continual respiratory therapy for for infants and small children and intracardiac repair in older children since repair in infancy does not relieve respiratory symptoms. We do not recommend a prosthetic pulmonary valve unless there is persistently elevated main pulmonary artery pressure.

Emphysema↗

Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.

Only two cases have been reported previously of the association of ventricular septal defect (VSD) with anomalous origin of the left coronary artery (ALCA) arising from the pulmonary artery. The purpose of this paper is to present two additional cases, to describe the pathophysiology, and to emphasize how the clinical course of this combination of defects differs from that of isolated ALCA. Patients with both of these anomalies present in infancy with manifestations only of a large left-right ventricular shunt and pulmonary hypertension. Initially the ALCA is well perfused from the high pressure in the pulmonary artery. In these instances in which the pulmonary artery pressure subsequently decreased because of spontaneous reduction in size of the VSD, the left coronary arterial system became less well perfused. Because of this decreased perfusion in association with the left ventricular myocardial stress initially caused by volume overload, myocardial ischemia and ultimately infarction occurred. Early identification and repair of the anatomic abnormality could prevent irreversible myocardial damage.

Coronary Vessel Anomalies↗