Richard Van Praagh

Bio: Richard Van Praagh is an academic researcher from Boston Children's Hospital. The author has contributed to research in topics: Great arteries & Tetralogy of Fallot. The author has an hindex of 44, co-authored 117 publications receiving 6067 citations. Previous affiliations of Richard Van Praagh include University of Cincinnati Academic Health Center & Northwestern University.

The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases.

Abstract: Based upon a series of 57 necropsy cases and upon the literature, it was found that there are two basic types of common aorticopulmonary (A-P) trunk: those with a ventricular septal defect, and those without. The latter is exceedingly rare and did not occur in this series. Truncus with a ventricular septal defect was found to consist of the following: (1) absence of the distal portion of the pulmonary infundibulum (very similar to tetralogy of Fallot with pulmonary atresia); (2) partial or complete absence of the pulmonary valve; (3) partial or complete absence of the aorticopulmonary septum; and (4) arterial arches 4 and 6 varied inversely in their development (e.g., well developed aortic arch with absent ductus arteriosus, or interrupted aortic arch with a large patent ductus arteriosus). Thus, there appeared to be no such thing as “true” persistent truncus arteriosus in the timehonored sense of persistence of an undivided conotruncal channel. While it is correct that the truncus is undivided (the aorticopulmonary septum being partially or totally absent), at the level of the conus the anomaly is not a failure of septation but absence of the entire distal portion of the pulmonary infundibulum (septum and free wall). Hence, at the level of the conus, common trunk is not a large infundibular septal defect but an extreme tetralogy of Fallot. At the distal conus, nothing has persisted. When the aortic arch was present, there was a high incidence of right aortic arch (27%) similar to tetralogy. The truncal valve basically was the aortic valve, explaining why it usually was tricuspid (67%). When some pulmonary leaflet tissue persisted, made possible by absence of the aorticopulmonary septum at the semilunar valve level, the truncal valve then was quadricuspid (24%). Failure of leaflet separation either in a basically tricuspid or quadricuspid valve occasionally resulted in a bicuspid truncal valve (7%). Truncus is not a form of transposition of the great arteries. Mitral-aortic fibrous continuity was present in all cases. The ventricular septal defect is not primarily “membranous.” It is due to absence of the distal pulmonary infundibulum. The coexistence of single ventricle and common aorticopulmonary trunk has been considerably overestimated. This combination did not occur in this series and is extremely rare (?non-existent). A revised and simplified classification of truncus is proposed.

Interrupted aortic arch: Surgical treatment

Abstract: A new palliative surgical procedure for interruption of the aortic arch was successfully performed in an infant. A Teflon graft was inserted from the main pulmonary artery to the descending thoracic aorta in order to bypass a stenotic ductus arteriosus, and both pulmonary artery branches were banded to reduce the pulmonary blood flow. This procedure may also prove life-saving in the hypoplastic left heart syndrome, with the addition of atrial septal defect creation when left atrial decompression is indicated. This syndrome was the commonest cause of death from congenital heart disease in the first month of life in our recent experience (31 percent), based on a consecutive autopsy series of 174 neonates with congenital heart disease. The anatomic findings in 10 autopsy cases of interrupted aortic arch are summarized. A new understanding of the morphogenesis of these anomalies is presented, based on correlation of embryologic and pathologic data. The literature on interruptions of the aortic arch is reviewed, and surgical experience with these malformations is considered in detail.

ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease

Abstract: PREAMBLE......e4 APPENDIX 1......e121 APPENDIX 2......e122 APPENDIX 3......e124 REFERENCES......e124 It is important that the medical profession play a significant role in critically evaluating the use of diagnostic procedures and therapies as they are introduced in the detection, management,

Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation

Abstract: Jaundice occurs in most newborn infants. Most jaundice is benign, but because of the potential toxicity of bilirubin, newborn infants must be monitored to identify those who might develop severe hyperbilirubinemia and, in rare cases, acute bilirubin encephalopathy or kernicterus. The focus of this guideline is to reduce the incidence of severe hyperbilirubinemia and bilirubin encephalopathy while minimizing the risks of unintended harm such as maternal anxiety, decreased breastfeeding, and unnecessary costs or treatment. Although kernicterus should almost always be preventable, cases continue to occur. These guidelines provide a framework for the prevention and management of hyperbilirubinemia in newborn infants of 35 or more weeks of gestation. In every infant, we recommend that clinicians 1) promote and support successful breastfeeding; 2) perform a systematic assessment before discharge for the risk of severe hyperbilirubinemia; 3) provide early and focused follow-up based on the risk assessment; and 4) when indicated, treat newborns with phototherapy or exchange transfusion to prevent the development of severe hyperbilirubinemia and, possibly, bilirubin encephalopathy (kernicterus).

Catheterization of the Heart in Man with Use of a Flow-Directed Balloon-Tipped Catheter

Abstract: Pressures in the right side of the heart and pulmonary capillary wedge can be obtained by cardiac catheterization without the aid of fluoroscopy. A No. 5 Fr double-lumen catheter with a balloon just proximal to the tip is inserted into the right atrium under pressure monitoring. The balloon is then inflated with 0.8 ml of air. The balloon is carried by blood flow through the right side of the heart into the smaller radicles of the pulmonary artery. In this position when the balloon is inflated wedge pressure is obtained. The average time for passage of the catheter from the right atrium to the pulmonary artery was 35 seconds in the first 100 passages. The frequency of premature beats was minimal, and no other arrhythmias occurred.

ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: Executive summary - A report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)

Abstract: It is important that the medical profession play a central role in critically evaluating the use of diagnostic procedures and therapies introduced and tested for detection, management, or prevention of disease. Rigorous, expert analysis of the available data documenting absolute and relative