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Biomedical subjects

R Van Praagh

Publications and source records attributed to R Van Praagh.

At least 73 records · Page 4Linked to original sources

Infundibular septal resection: surgical anatomy of the superior approach.

A technique for extensive resection of the infundibular septum through the superior transarterial approach is proposed. Infundibular septal resection facilitates intraventricular rerouting for anatomic correction of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle. This technique also may be used to enlarge progressively obstructive but physiologically advantageous ventricular septal defects, for example, with tricuspid atresia. Through the superior transaortic or transpulmonary approach, the landmarks of the infundibular septum are the intercoronary commissure of the aortic valve and the septal commissure of the pulmonary valve, both of which are directly above the middle of the infundibular septum. With this exposure, the infundibular septum may then be resected easily and relatively completely and a large opening created immediately beneath the semilunar valves. The feasibility of infundibular septal resection by the superior approach was assessed post mortem in 25 cases of transposition of the great arteries with ventricular septal defect and in 20 autopsied cases of double-outlet right ventricle. This technique was subsequently applied successfully to enlarge the obstructive ventricular septal defect of a 2-year, 11-month-old girl with D-transposition of the great arteries. The advantages of the proposed technique include technical ease, relative completeness of infundibular septal resection, and the facilitation of intraventricular repair of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle.

Adolescent↗

Diagnosis of complex congenital heart disease: morphologic-anatomic method and terminology.

A summary and brief illustration of the morphologic-anatomic method of diagnosis of congenital heart disease is presented. The principles of scientific neologizing are considered and exemplified. Scientific freedom of speech and expression is commented upon. It is suggested that unnecessary renaming of numerous cardiac anatomic structures and many forms of congenital heart disease be discontinued, and that terminology be de-emphasized.

Heart↗

Aristotle's "triventricular" heart and the relevant early history of the cardiovascular system.

Aristotle said that the human heart has three ventricles--right, left, and middle--a concept that has often been viewed as an astonishing error. But was it? Aristotle did not miscount ventricles. In the third century BC, all cardiac chambers were called "ventricles," meaning "cavities." The "ears" (auricles) were distinguished from the "cavities" (ventricles) by Herophilus of Alexandria (c 300 BC) and by Rufus and Ephesus (a contemporary of Jesus Christ). Aristotle regarded the right atrium as a venous dilatation, not as a part of the heart. Aristotle's "right ventricle" was our right ventricle. His "left ventricle" was our left atrium. His "middle ventricle" was our left ventricle. Because he did not count the right atrium, Aristotle considered the human heart to be three-chambered or "triventricular," consisting of the right ventricle, the left atrium, and the left ventricle. This report summarizes the relevant early history of the cardiovascular system.

Anatomy↗

Origin of pulmonary artery branch from ascending aorta. Primary surgical repair in infancy.

1. Surgical repair of origin of a pulmonary artery branch from the ascending aorta should be performed as early as possible in order to prevent death from congestive heart failure or the development of irreversible pulmonary vascular obstructive disease. 2. Deep hypothermic circulatory arrest greatly facilitates surgical repair of this lesion in infancy, as is illustrated by the three cases reported herein. 3. Direct anastomosis of the ectopic pulmonary artery branch to the main pulmonary artery is the surgical technique of choice. 4. Origin of the RPA or the LPA from the ascending aorta results from origin of the RPA or LPA from the aortic sac, instead of from the confluent sixth arches. Typically, the RPA has failed to migrate leftward because of abnormal development of the wall of the aortic sac, abnormal development of the sixth arches, or both. 5. Origin of the RPA or the LPA from the ascending aorta should be distinguished from origin of the "RPA" or of the "LPA" from the innominate artery or from the aortic arch via a PDA or a collateral artery.

Cardiac Catheterization↗

What is a ventricle? The single-ventricle trap.

Whether a chamber is or is not a ventricle is determined by its myocardial morphological characteristics, not by the entering atrioventricular (AV) valves. The anatomic characteristics of the morphologically right ventricle and the morphologically left ventricle have been well described, are widely understood, and do not need to be changed. What a ventricle is should not be redefined in terms of the AV connections because such redefinition is based on the classic definition of single ventricle, which itself is not satisfactory. This old definition is wrong in principle, violates the morphological method of diagnosis and designation of the cardiac principle, violates the morphological method of diagnosis and designation of the cardiac chambers, and has two kinds of exceptions. Consequently the classic definition of single ventricle must not be used as a paradigm (model) for the redefinition of what a ventricle is or is not. The approach and terminology based on the use of the unsatisfactory single-ventricle is or is not. The approach and terminology based on the use of the unsatisfactory single-ventricle paradigm approach and terminology based on the use of the unsatisfactory single-ventricle paradigm should be discontinued, eg, "primitive ventricle," "main chamber," "accessory chamber," "trabecular pouch." Being unnecessary, these nonmorphological components of the terminology of congenital heart disease may be omitted. Morphological anatomy, unadorned, is the key to clarity, simplicity, and accuracy.

Heart↗

Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves.

In 3 patients with absent pulmonary valve syndrome and absent ductus arteriosus, the lungs were injected and analyzed postmortem using morphometric techniques. Two patients had tetralogy of Fallot and 1 had D-transposition of the great arteries, the latter being the first autopsy-proved case of absent pulmonary valve with transposition. In addition to the expected dilatation of the central pulmonary arteries and compression of the mainstem bronchi, postmortem pulmonary arteriography revealed a bizarre pattern of hilar branching. Instead of single segmental arteries, tufts of arteries arose which entwined and compressed the intrapulmonary bronchi. In all 3 patients the histologic structure of the pulmonary arteries was abnormal. The elastic lamina of the media of the right and left pulmonary arteries were increased in number outside the lung, but were decreased within the lung. At both sites, the elastic laminae were thickened and fragmented. In the 2 ventilator-dependent patients, there was slight medial hypertrophy and extension of muscle into normally nonmuscular arteries. In 1 of the 2 cases in which the number of bronchial generations was counted, they were decreased, and in the 1 case in which bronchial count was unknown, alveolar multiplication was severely impaired. Therefore, our data may explain why, in some patients with absent pulmonary valve syndrome, relief of compression of the mainstem bronchi alone does not appreciably alleviate or reverse severe respiratory disease.

Bronchi↗

Potentially parachute mitral valve in common atrioventricular canal: pathological anatomy and surgical importance.

An essentially single focus of left ventricular chordal insertion was found in 23 of 164 autopsied cases of common atrioventricular canal (CAVC) with a normally formed spleen (14%). Suture closure of the cleft of the mitral valve in such cases results in the surgical creation of parachute mitral valve, often with fatal iatrogenic mitral stenosis. In such patients, the cleft of the mitral valve is its main orifice and must not be sutured closed. There are four anatomic types of potentially parachute mitral valve in CAVC: (1) with one papillary muscle group and one mitral orifice (type 1A), in 10 cases (43%); (2) with one papillary muscle group and two mitral orifices (type 1B), in one case (4%); (3) with two papillary muscle groups and one mitral orifice (type 2A), in five cases (22%); and (4) with two papillary muscle groups and two mitral orifices (type 2B), in seven cases (30%). Parachute mitral valve exists only when the AVC is divided, either naturally or surgically. The essence of parachute mitral valve is an essentially single focus of chordal insertion. One or both left ventricular papillary muscle groups may be present. The presence of only one focus of left ventricular chordal insertion contraindicates cleft closure.

Adolescent↗

Selective coronary arteriography in congenitally corrected transposition of the great arteries.

Three cases of congenitally corrected transposition of the great arteries in adults who underwent selective coronary arteriography are presented. The morphologic features of the epicardial coronary anatomy are distinctive and are identifiable angiographically as morphologically right and left coronary arteries that are specifically concordant with the morphologically right and left ventricles. This relation is constant in the presented cases, in previously published coronary arteriograms of congenitally corrected transposition of the great arteries and in a review of the anatomic studies of congenitally corrected transposition of the great arteries that identify the coronary arterial pattern. Thus the angiographic characteristics of the epicardial coronary arterial pattern permit identification of the morphologic features of the underlying ventricle regardless of other spatial relations.

Adult↗

Unusual vascular anomalies causing persistent pulmonary hypertension in a newborn.

A unique case of pulmonary vascular anomalies causing persistent pulmonary hypertension in a newborn is described. The child died 3 days after birth. Necropsy revealed marked hypoplasia of the right and left pulmonary arteries with a normal main pulmonary artery, patent ductus arteriosus, bilateral systemic arteries to the lungs from the abdominal aorta, and partial anomalous pulmonary venous connection. Quantitative morphometric techniques demonstrated slight abnormalities of alveolar development and severe arterial medial hypertrophy with abnormal extension of muscle into small peripheral arteries. Bronchopulmonary development appeared relatively normal in spite of the vascular abnormalities.

Angiocardiography↗

Electrophysiologic delineation of the specialized atrioventricular conduction system in two patients with corrected transposition of the great arteries in situs inversus (I,D,D).

Electrophysiologic delineation of the atrioventricular conduction system at surgery is described in two patients with corrected transpostion of the great arteries in situs inversus. Intra-atrial electrograms were recorded in one patient from sites immediately adjacent to the coronary sinus located in the left-sided right atrium. The intraventricular portion of the atrioventricular conduction system was identified in both patients along the posterior and inferior margin of the ventricular septal defect, in contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. In contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. The course of the conduction system in the hearts of these two patients and a possible relationship to the cardiac loop and dual origin of the atrioventricular node is discussed. These cases illustrate the usefulness of segmental diagnosis of congenital heart disease and of electrophysiologic identification of the specialized atrioventricular conduction system at surgery.

Adolescent↗