Gordon M. Folger

The scimitar syndrome. Anatomic, physiologic, developmental and therapeutic considerations.

Division of Pediatric Cardiology, Henry Ford Hospital, Detroit, Michigan and the Division of Pediatric Cardiology, Medical College of Georgia, Augusta, Georgia .... I inclose herewith an account of a case which fell under my own notice, of transposition of the heart, and imperfect development of the right lung, while the vessels and viscera of the abdomen retained their usual situations. If you think it sufficiently interesting for insertion in your valuable journal, it is much at your service. I have the honour to remain, sir,

Hypoplastic left heart syndrome: Clinicopathologic and hemodynamic correlation

Abstract Eleven patients with the hypoplastic left heart syndrome, defined as atresia of the aortic or mitral valve, or both, with normally related great arteries and intact ventricular septum, were studied. There were 2 clinical groups. Group I (8 cases) comprised patients with early distress, ashen cyanosis and feeble or absent peripheral pulses in the presence of a hyperdynamic precordium and death in the first week of life. Group II (3 cases) comprised patients with good peripheral pulses, marked cyanosis and survival for more than 1 week. All of the patients had characteristic electrocardiographic and radiographic findings. In Group I, values for pulmonary oxygen saturation were elevated with pressures moderately increased, whereas in Group II, oxygen saturation values were greatly lowered with markedly increased pressures. All patients had hypoplastic ascending aorta, large patent ductus arteriosus and diminutive left ventricle, left atrium and foramen ovale. Three of 8 had abnormalities of the coronary sinus. A positive correlation can be made between the clinical appearance and the hemodynamic findings which may allow selection for palliative surgical procedures.

Annular subvalvular left ventricular aneurysm in a North American infant

Abstract A case of suspected annular submitral left ventricular aneurysm in a 6-month-old Negro infant is reported and the pertinent literature reviewed. The occurrence of this abnormality in an individual of this age as well as race indicates a developmental origin of the disease entity.

Acute Rheumatic Fever in an Arabian Gulf Country Effect of Climate, Advantageous Socioeconomic Conditions, and Access to Medical Care

An eleven-year study of the incidence and consequences of acute rheumatic fever was carried out in a country in which a uniform climate together with national characteristics of insularity, wealth, and unrestricted access to free medical care contribute prominently to the epidemiologic milieu. Study subjects were 86 children, aged four to fourteen years, satisfying criteria for acute rheumatic fever. Study methods included clinical evaluation, standard laboratory studies, and echocardiography. A declining incidence of rheumatic episodes, ranging from 1.06 to 18.6/100,000 population (average 11.2/100,000), was identified. The course of the episode was generally mild. Arthritic findings predominated (92%), followed by carditis identified clinically in 43% and, with the addition of echocar diography, in 71%. Residual valvular regurgitation, as a longer term consequence, persisted in 46% of those with auscultatory confirmation of valvulitis. No recurrences were identified. Comparison with countries of similar socioeconomic status revealed rela tively unimportant differences. Comparison with nearby disadvantaged countries identi fied striking contrast. It may be concluded that among the contributing factors, for the improvement in the incidence and sequelae of a rheumatic episode, are an advantaged socioeconomic environment and accessibility to unlimited medical care.

Supravalvular tricuspid stenosis: Association with developmental abnormalities of the right heart and derivatives of the sixth aortic arch☆

Abstract Two patients with the combination of supravalvular obstruction of the tricuspid orifice associated with abnormalities of the pulmonary valve, pulmonary artery and the ductus arteriosus are described. Previously reported instances of these malformations are reviewed and the proposed embryologic mechanisms discussed. Recognition of the tricuspid obstruction prior to surgical correction of the associated malformations may be made by angiocardiography. If the abnormality is not diagnosed, as in the 2 patients herein described, surgery may be directed only at the associated anomalies, leaving the tricuspid obstruction unaltered.

Nodular fibroelastosis (Fibroelastic hamartoma) A tumorous malformation of the heart

Abstract A tumorous malformation, i.e., nodular fibroelastosis of the left ventricle and interventricular septum of the heart, is described. A relation between the tissue composing the mass and that seen with endocardial fibroelastosis is postulated. The clinical and laboratory findings bear distinct similarities to those of previously reported cases and suggest specificity for this type of malformation. Inclusion of this abnormality in the group of cardiomyopathies is recommended.

Tricuspid atresia with transposition of the great vessels

Diagnostic and therapeutic experience with patients having tricuspid atresia with transposition of the great vessels is reviewed and contrasted with previous reports of this condition. In the absence of pulmonic stenosis, the clinical picture is nearly indistinguishable from massive left-to-right shunts of all causes, although the electrocardiogram and cardiac roentgenogram usually indicate the correct diagnosis. The presence of naturally occurring pulmonary stenosis markedly alters the clinical picture; surgical creation of pulmonic stenosis appears to be lifesaving.

Suppurative Pericarditis Review of the Problem Plus Observations on Two Patients

Think of suppurative pericarditis in in stances of fever of unknown origin—it is a treatable disease if recognized before the terminal events. Suppurative pericarditis is still with us despite the wider use of antibiotics. These two recent cases serve to remind the prac titioner of the details of diagnosis and management of this persistent pediatric problem.

Acidemia of Cardiogenic Origin in Young Infants with Cyanotic Congenital Heart Abnormalities: Pathogenesis, Diagnosis and Treatment, with Particular Emphasis on Neonatal Acid-base Disturbance

* Formerly Associate Professor, Department of Pediatrics, Medical College of Georgia, Augusta, Georgia. Present Position, Director, Section on Pediatric Cardiology, Henry Ford Hospital, Detroit, Michigan 48202. METABOLIC acidemia of the newly born secondary to hypoxemia is a common lifethreatening event which, for its importance and relative ease of management, has received little comment. Particularly notable, however, are the reports of Gootman et al.1 and of Rudolph implicating the role of lactic acid in its genesis. Simply stated, the discrepancies between the supply of oxygen and the metabolic requirements of the cells for oxygen underlie the entire problem and form the bases for treatment. In infants with cyanotic heart disease, this complication is the most common cause of death, far surpassing heart failure and cyanosis, per se. This discussion considers two aspects of care, each equally important, and emphasizes the immediate or emergency treatment of these extremely ill infants. For a full understanding of this metabolic acidemia, how-

The Management of Valvular Heart Disease in Children

The child with significant valvular heart disease may present in any single instance both the most difficult of management decisions or a surprisingly simple one. Thus, if a direct surgical approach on the valve is feasible, the problems may very well be quite simple and straightforward; conversely, if valve replacement is indicated such may not be the case. We have recently reported our experience with one type of valvular heart condition, congenital aortic stenosis, in respect to determination of surgical approach based upon the ability to predict the orifice area and valvular anatomy.1,2 This report reviews our current experience with this technique. As well, the experience concerning the approach to each of the valvular conditions will also be reviewed with emphasis in respect to the left heart valves and particularly their long term postoperative management.