50th anniversary historical article. Congenital heart disease.
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Female↗
Biomedical subjects
M A Engle
Publications and source records attributed to M A Engle.
Late follow-up after venous switch operation (Mustard procedure) for simple and complex transposition of the great arteries.
Most adolescents and young adults born with complete transposition of the great arteries (TGA) and alive today are survivors of the Mustard operation. This study reports on the serial, long-term (from 10 to > 20 years) follow-up of 85 patients who underwent this operation between 1971 and 1981. Of these, 63 had simple and 22 complex TGA. The age at surgery ranged from 2 days to 17 years. The early mortality rate was 10.5% and the late mortality 9.2%. The actuarial survival rate after 15 years was 86% for simple and 64% for complex TGA. Event-free survival after 15 years was 77% for simple and 46% for complex TGA. Yearly review of electrocardiograms and, less frequently, of Holter tracings disclosed a lower mean resting heart rate and decrease over time in sinus rhythm and an increase in active arrhythmias. Fifty-two percent had resting sinus rhythm and 17% had active arrhythmias at 16 to 20 years of follow-up. Exercise stress testing in 21 patients revealed resumption of sinus rhythm during exercise but significant diminution of endurance time and peak heart rate response. Seven of the survivors (9.2%) required reoperation. Of these, 4 had severe tricuspid regurgitation following patch closure of ventricular septal defect. This study shows gratifying long-term and event-free survival for the majority of patients who underwent surgery by this venous switch procedure.
Actuarial Analysis↗
Right and left ventricular performance 10 years after Mustard repair of transposition of the great arteries.
Twenty-two late survivors of Mustard repair of transposition of the great arteries underwent treadmill exercise testing to assess exercise endurance, and radionuclide cineangiography to measure rest and exercise right ventricular (RV) and left ventricular (LV) ejection fraction (EF). Mean age at Mustard repair was 1.8 +/- 2.4 years and at initial exercise study 14.1 +/- 4.5 years. All patients were asymptomatic at study entry. Treadmill endurance time (9 to 12 minutes) was within the normal range for age in 18 of 20 patients. LVEF and RVEF at rest were within normal limits (55 +/- 8% and 52 +/- 9%, respectively). All LVEFs and RVEFs during exercise were also within the normal range (64 +/- 8% and 57 +/- 9%, respectively). However, the magnitude of increase in EF (rest to exercise) was subnormal for the right ventricle in 7 patients and for the left ventricle in 2 patients. Exercise RVEF was higher in patients with simple transposition who underwent repair at < 1 year of age than in those who underwent operation at age > 1 year (62 +/- 10% vs 52 +/- 7%). Serial study in 6 patients revealed no change in average RVEF or LVEF after an average interval of 4.4 years. One patient with complex transposition subsequently developed refractory congestive heart failure requiring cardiac transplantation. It is concluded that exercise endurance and LVEF and RVEF at rest and exercise are generally well preserved up to 19 years after Mustard repair, particularly in cases of simple transposition of the great arteries repaired before 1 year of age.
Adolescent↗
Managed care involvement by cardiovascular specialists: prevalence, attitudes and influence on practice.
OBJECTIVES: The purpose of this study was to determine the involvement in and attitudes toward managed care by cardiovascular specialists and the influence of such programs on their practices. BACKGROUND: No in-depth study has measured the impact of managed care on cardiovascular specialists. Therefore, we conducted a mail survey to determine the prevalence of managed care arrangements among cardiovascular specialists and variations among pediatric and adult cardiologists and cardiovascular surgeons; the types of managed care arrangements in which cardiovascular specialists are engaged; the reasons why those not participating in managed care have chosen not to do so; and the general attitudes among cardiovascular specialists with regard to various aspects of managed care. In addition, we evaluated the impact of managed care among several aspects of cardiovascular practice. METHODS: A questionnaire was mailed in the spring of 1993 to 4,577 practicing, domestic, American College of Cardiology (ACC) members selected at random from within each primary cardiovascular specialty group (adult cardiologists, pediatric cardiologists and cardiovascular surgeons). Additional data concerning practice characteristics were cross tabulated using results from the 1992 ACC membership profile survey. RESULTS: In total, 1,961 of the 4,577 members responded to the survey, representing a 43% response rate. Of all survey respondents, 76% reported entering into at least one relationship with a health maintenance organization (HMO) or preferred provider organization (PPO). Of those not participating in managed care arrangements, the most frequently mentioned reason was "concern over the quality of care." This reason was cited by 51% of those not entering into HMO relationships and 41% of those not participating in PPOs. The majority of respondents indicated that they do not strongly object to the gatekeeper approach to managing nonemergent patients, although more than half indicated concern that gatekeepers may not be appropriate in the management of cardiac emergencies. In addition, cardiovascular specialists report that under managed care, referrals have not increased, income has decreased, and managed care formularies have not substantially affected their ability to prescribe appropriate medication to their patients. CONCLUSIONS: Despite concerns over the quality of care and contract requirements and general philosophical opposition of cardiovascular specialists, most are becoming integrated into managed care environments.
Adult↗
Glutaraldehyde treated autologous pericardium in complete repair of tetralogy of Fallot.
Pericardium has been used for decades to facilitate the repair of tetralogy of Fallot (TOF). The impact of glutaraldehyde preserved autologous pericardium when used as a right ventricular outflow tract (RVOFT) patch in TOF was analyzed in 36 consecutive children undergoing complete transventricular repair. In 18 (group I) the pericardium was treated in conventional fashion (harvested and preserved in saline solution). In the other patients, the pericardium was treated in 0.625% glutaraldehyde solution for 20 minutes and then washed in saline prior to being implanted as an RVOFT patch (group II). The perioperative (prior to hospital discharge) as well as the 6-month postoperative Doppler echocardiograms were assessed with 100% follow-up. The studies were evaluated and graded by blinded observers for the presence and severity of an RVOFT dilatation (+0 to +4) relative to the size of the aortic valve annulus. The age of patients in group I and group II (29 months, 34 months) were similar as was the incidence of transannular patching (44%, 41%). Postrepair hemodynamics revealed no significant difference in the right ventricular/left ventricular pressure ratios (42%, 41%) or in the systolic RVOFT pressure (24 mmHg, 29 mmHg). The predischarge echocardiograms showed no outflow tract (OFT) dilation in either group. There was no morbidity or mortality in either group. At approximately 6 months postoperatively (6.2 mo, 5.7 mo), 72% of group I patients had RVOFT diameters that were larger (+1, +2) than the predischarge diameters when reviewed by three observers. In group II there was a single patient with dilatation of the RVOFT and this graded as +1.(ABSTRACT TRUNCATED AT 250 WORDS)
Blood Pressure↗
A five-year clinical experience with 112 Blalock-Taussig shunts.
In spite of recent advances in neonatal open repair for complex cyanotic heart disease, some patients require palliation with a systemic-to-pulmonary artery shunt. We report a 5-year experience (1985-1990) with 112 Blalock-Taussig shunts. Forty-six of the 92 patients had some variant of tetralogy of Fallot, with a wide spectrum of diagnoses in the remainder. The median age at surgery was 3 months. A classic Blalock-Taussig shunt was done in 26% (group I), and a 4- or 5-mm PTFE graft was utilized in the remainder (group II). The technical aspects of each of the procedures are reviewed. There were three early deaths in the entire group, none of them related to Blalock-Taussig shunt function. There was no incidence of early shunt insufficiency, bleeding, infection, limb ischemia, or pulmonary artery distortion. There was a 21% incidence of clinical congestive heart failure, seen somewhat more commonly in group I. The overall need for reshunting/open repair was similar in both groups, but there was a statistically longer interval between the initial Blalock-Taussig shunt and the second procedure in group I (21.6 vs 12.4 months). The Blalock-Taussig shunt remains a safe, reliable, and effective means of increasing pulmonary flow.
Adolescent↗
Transient complete heart block following percutaneous balloon pulmonary valvuloplasty: treatment with systemic corticosteroids.
Balloon pulmonary valvuloplasty is a safe and effective treatment for congenital pulmonic valve stenosis. This report describes a child who developed complete atrioventricular (AV) block following balloon pulmonary valvuloplasty. The child was treated with a 10-day course of systemic corticosteroids. The heart block gradually resolved during the first week following the procedure and has not recurred. Heart block is a known complication of right heart catheterization and has been described following balloon pulmonary valvuloplasty. Systemic corticosteroids have been used to treat AV block of various etiologies and may have contributed to the child's recovery in this case.
Catheterization↗
Development of obstruction to ventricular outflow and impairment of inflow in glycogen storage disease of the heart: serial echocardiographic studies from birth to death at 6 months.
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Cardiomyopathy, Hypertrophic↗
Congenital aortic regurgitation: natural history and management.
OBJECTIVES AND BACKGROUND: Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no other cardiac lesions who had congenital valvular aortic regurgitation. METHODS: From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. RESULTS: In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. CONCLUSIONS: Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.
Aortic Valve↗
Cardiac issues in the pediatric emergency room.
A great variety of problems referable to the cardiovascular system can prompt a visit to the pediatric emergency room. From the initial presentation of congenital heart disease, to the subsequent life-long management of these patients, to miscellaneous problems like Kawasaki disease and chest pain, the front-line pediatrician must be skilled in the recognition and early management of myriad complaints. This article focuses on information that can assist the emergency pediatrician in the evaluation and treatment of the cardiac patient from arrival in the emergency room until transfer of care to the pediatric cardiologist or inpatient staff.
Acute Disease↗
Endocarditis with aneurysm involving an aortic homograft used to correct a truncus arteriosus: medical-surgical salvage.
An aneurysm of an aortic homograft conduit, used to correct a type I truncus arteriosus anomaly in a four month old infant, developed when the patient was 15. Blood cultures grew Staphylococcus aureus. The aneurysm was detected by magnetic resonance imaging and digital subtraction angiocardiography. An emergency open heart operation, guided by these investigations, was performed to remove the original homograft and replace it with another valved aortic homograft. Postoperative antibiotic treatment had to be stopped when profound neutropenia developed. This responded to treatment with recombinant human granulocyte colony stimulating factor. Three years later she was symptom free and did not require medication. Chest x rays and echocardiograms showed a normally functioning heart and conduit valve.
Aortic Aneurysm↗
Unique variant of Taussig-Bing heart: double-outlet right ventricle with double ventricular septal defects and double overriding of great arteries.
A cyanotic, tachypneic newborn was diagnosed to have double-outlet right ventricle of the Taussig-Bing type. Cardiac failure did not respond to medical treatment or surgical palliation. Postmortem examination revealed two ventricular septal defects (VSDs), one a malalignment VSD in the membranous septum and adjacent tissue and the other in the anterosuperior part of the muscular septum. The D-malposed aortic root emerged mainly from the right ventricle, with aortic-mitral continuity. The larger posterolateral pulmonary root arose almost entirely from the right ventricle, confluent with the muscular VSD, and unrelated to the mitral valve. Its right ventricular aspect was obstructed by hypertrophied infundibulum. This unique malformation of the heart functioned as a double-outlet right ventricle of Taussig-Bing type. In addition, however, the malformation had elements of tetralogy of Fallot because of the malaligned VSD and hypertrophied conal musculature (although pulmonary flow was excessive), and also of complete transposition of the great arteries because of the arrangements of the two VSDs, which favored aortic flow from right ventricle and pulmonary blood flow from the left ventricle. Thus, a single heart presented similarities to three anatomic and functional entities.
Double Outlet Right Ventricle↗
Successful coil embolization for diffuse, multiple pulmonary arteriovenous fistulas.
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Adult↗
Prolonged survival in patients with beta-thalassemia major treated with deferoxamine.
To determine whether survival of patients with beta-thalassemia major has been prolonged by management that utilizes hypertransfusion and chelation with deferoxamine, we analyzed longevity by the Kaplan-Meier product-limit method. Group 1 patients (n = 71) followed between 1960 and 1976 with a low-transfusion regimen (pretransfusion hemoglobin level 7 to 8 gm/dl) and no chelation had an estimated median age of survival of 17.4 years, whereas it was 31.0 years for group 2 subjects (n = 80), who began hypertransfusion between 1976 and 1978 (pretransfusion hemoglobin level 10.5 to 11.5 gm/dl) and chelation with deferoxamine (20 to 60 mg/kg per day) (p less than 0.0001). For 70 patients who were treated with hypertransfusion and deferoxamine, we had data to calculate the ratio of total milligrams of transfusional iron to cumulative grams of deferoxamine. The 24 patients who died had a total iron burden of greater than 1.05 gm/kg; the ratio for them exceeded 31. These patients were characterized by poor compliance with chelation or by late start of therapy, with inability to receive enough deferoxamine before death. Death was preceded by arrhythmia requiring therapy in all but one, and by cardiac failure in all. Of 41 similarly iron-loaded survivors, 33 had a ratio of less than 31; only three had an arrhythmia, and five had cardiac failure. We conclude that treatment with deferoxamine, when used in amounts proportional to iron burden, delayed cardiac complications and improved longevity.
Adolescent↗
The heart and conduction system in acute Kawasaki disease: report of fraternal cases--one lethal, one relapsing.
The heart and conduction system were studied in a 4 1/2-year-old boy with Kawasaki disease who died suddenly in the early phase of illness. Severe perivasculitis, neuritis, pancarditis, and involvement of all parts of the specialized conduction system were found. The boy's brother, born a year later, at the age of 12 months had relapsing Kawasaki disease. He received intravenous gamma globulin and recovered.
Aorta↗
Pulmonary function in preterm infants following treatment with intravenous indomethacin.
Pulmonary function tests, including measurements of arterial blood gas levels, total pulmonary compliance, and arterial-alveolar oxygen ratios, were performed in 38 ventilator-dependent preterm infants with respiratory distress syndrome who weighed less than 1500 g at birth. Twenty-seven had a physiologically significant patent ductus arteriosus (PDA). Twelve were assigned at random to receive three doses of intravenous indomethacin, 0.2 mg/kg per dose, on the fourth day of life. This treatment resulted in ductal closure in seven infants by the seventh day of life. Another concurrently observed group of 15 infants with PDA received no indomethacin. A third group of 11 infants lacked evidence of a PDA. Pulmonary function in the infants who received indomethacin did not differ significantly from that in the other two groups.
Ductus Arteriosus, Patent↗
Clinical trial of single-dose intravenous gamma globulin in acute Kawasaki disease. Preliminary report.
Gamma globulin administered in a single dose of 1 g/kg of body weight intravenously caused prompt clinical improvement in 27 of 32 consecutive children with Kawasaki disease treated by the 12th day of illness. Response was equally good for the 20 children treated in the first week and the 12 treated in the second week. Fever and clinical signs abated within the first day after treatment, the mean white blood cell count normalized by 48 hours, and the sedimentation rate continued to be elevated for about 2 weeks, while the platelet count rose during the first 2 weeks after treatment and returned to normal approximately 1 month after treatment. Five children with incomplete relief needed more than the single dose before resolution of signs and symptoms occurred. Coronary aneurysms in 2 patients before treatment regressed by 2 weeks. No patient developed coronary aneurysms. No child had sequelae of Kawasaki disease at a follow-up of 2 to 31 months. We believe that although this was a one-arm, uncontrolled pilot study, the results suggest that this protocol provides a safe, flexible, and effective treatment for acute Kawasaki disease.
Acute Disease↗
Growth and development of state of the art care for people with congenital heart disease.
For 40 years the American College of Cardiology has been a responsible organization in promoting the art and science of cardiac care for people of all ages. This review chronicles the leapfrogging of medical and surgical creativity and contributions to saving lives and making those lives healthy through informed care for people with congenital heart disease.
Cardiac Surgical Procedures↗