From Maude to Claude: the musings of an insomniac in the era of evidence-based medicine

Abstract

nurtured this interest and became my mentors. I remember so well the reading list they suggested to provide a firm and solid background in paediatric cardiovascular medicine: Helen Taussig's two volume text 1 (Fig. 1); Alexander Nadas' Pediatric Cardiology 2 (Fig. 2); and the book emanating from the Karolinska Institute, of which Edgar Mannheimer was a co-editor 3 (Fig. 3). I devoured those wonderful books, savoring the clinical descriptions. I was particularly intrigued even then by the wonderful angiography displayed in the text from the Karolinska Institute. A senior member of the Department of Pathology at Los Angeles, Dr. Harrison Latta, was my immediate supervisor when I decided to devote a year's study while still a medical student to pediatric cardiovascular pathology. After performing an autopsy on a baby with complex and mystifying congenital heart disease, Dr. Latta suggested that I write Dr. Jesse Edwards for his opinion. That was the beginning of a friendship extending over more than 30 years with one of the signal contributors to our understanding of the

The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

“…135,138,140 We suggested some years ago, in the Mannheimer lecture, that the development of pulmonary arteriovenous malformations following the classic cavopulmonary shunt or bidirectional cavopulmonary shunt is a wonderful paradigm for bed-to-bench research. 141 Such observations have now evolved, especially in the past decade, from those of a clinical nature or concern to resonate in more fundamental or basic investigations. This evolution was certainly stimulated in large part by the observations of Srivastava et al, with their appreciation of the development of pulmonary arteriovenous malformations secondary to hepatic venous exclusion, 66 and also from observations in the liverlung interface.…”

Designing therapeutic strategies for patients with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs

“…In those patients with previous banding and a restrictive ventricular septal defect, myocardial hypertrophy can be impressive, as the ventricular afterload is very much increased (Figs 2 and 3). 24,33,[35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50] The other concern about and the subsequent development of important obstruction in the systemic ventricular outflow tract, of course, is that this increased pressure and blood flow is transmitted to the pulmonary circulation, possibly jeopardizing the outcome of creation of the Fontan circulation. 42 We suggested nearly three decades ago several reasons for the "spontaneous" diminution in the size of the ventricular septal defect in these patients, 35 extending these observations in numerous publications.…”

“…42 We suggested nearly three decades ago several reasons for the "spontaneous" diminution in the size of the ventricular septal defect in these patients, 35 extending these observations in numerous publications. 12,15,24,29,33,36,37,[39][40][41]44,50 Firstly, in those patients without obstruction to the pulmonary outflow tract, the ventricular septal defect tended to be small, often smaller than the aortic root, and thus a defect that was predisposed to spontaneous diminution in size. This notion has subsequently been confirmed by others.…”

“…52 In the years before the conception of the Fontan circulation, and in the first part of the 1970s, it was the usual situation that the band was left in place for several years or more. 12,15,24,29,33,[35][36][37][39][40][41]44,50 Indeed, we and others cautioned about the role of banding as the initial palliation for these patients. 35,50,53,54 Others took issue with this.…”

“…This results in obstruction of the systemic ventricular outflow tract, an anatomical feature commonly identified as a risk factor for poor outcome. 7,10,12,33,34,[38][39][40][41][42][43][44][45]49,54,[56][57][58][59][60]179,180 In many of these patients, there is a common thread, specifically earlier banding of the pulmonary trunk. Even to this day, the precise definition of a restrictive ventricular septal defect remains contentious.…”