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Obstructing ventricular septal defect in double outlet right ventricle
LETTERS TO THE EDITOR
of our study, I soon realized that we had no clear idea what the big picture was. Had the pathologic findings changed from year to year over the past decade? We had no idea. Then we gradually realized that no one else seemed to know either! The large literature on the pathology of congenital heart disease consisted either of anecdotal case reports or of selected series--"numerators" without "denominators." This is why there are no pathologic findings, of which we are aware, with which our data can be compared. In this sense, we hope that our paper will begin a new genre inthe literature concerning the pathology of congenital heart disease. Similar studies from other centers are needed before it will be possible to see our findings concerning the postoperative pathology of congenital heart disease in perspective. These are the major reasons why we attempted to stress the enormous importance of reporting data and results in a complete and unselected way. Control series and statistical analysis in scientific publications are now generally understood to be desirable. But the need for completeness in reporting data and results is still largely unappreciated. Consequently, it is often not possible to ascertain the overall resuits for a given type of congenital heart disease at a particular institution. Moreover, it usually is not possible to compare, in a meaningful way, the surgical results in a given anomaly at several different institutions. This is why we suggested that the reporting of data and results should be complete and unselected. We think that this is a principle of basic importance and we hope that authors, reviewers and editors will take note. Application of this simple principle will considerably improve both the scientific quality and the practical usefulness of the medical literature. Richard Van Praagh, MD, FACC Children's Hospital Medical Center Harvard Medical School Boston, Massachusetts
OBSTRUCTING VENTRICULAR SEPTAL DEFECT IN DOUBLE OUTLET RIGHT VENTRICLE
countered three patients with an obstructive ventricular septal defect 2,:~ in our series of approximately 60 cases of double outlet right ventricle. The diagnosis of obstructive ventricular septal defect has important surgical implications because it may be necessary to enlarge the defect to ensure satisfactory postoperative hemodynamics. Also, surgical techniques may have to vary according to the type of obstruction, whether muscular or fibrous. 3 Preoperative diagnosis of the two varieties may be obtained by selective angiocardiography. Maria Serratto, MD, FACC Department of Pediatric Cardiology Cook County Children's Hospital Chicago, Illinois References 1. Srldaromont S, Feldt RH, Ritter DG, el al: Double outlet right ventricle: hemodynamic and anatomic correlations. Am J Cardio138:85-94, 1976 2. Serratto M, Arevalo F, Goldman EJ, el al: Obstructive venlricular septal defect in double outlet right ventricle. Am J Cardiol 19:457-643, 1967 3. Pellegrlno PA, Eckner FA, Meier MA, el al: Double outlet right ventricle with f~romuscular obstruction to left ventricular outlet. J Cardiovasc Surg 14:253-260, 1973
REPLY
We have now observed an obstructing ventricular septal defect in 3 of a total of 72 patients with double outlet right ventricle with levocardia. Two of those with significant ventricular septal defect obstruction demonstrated by pressure measurement were not part of our previous report because full hemodynamic data were not available. We agree that the diagnosis of obstructive ventricular septal defect has important implications. It is necessary, under those circumstances, for the surgeon to enlarge the ventricular septal defect when the diameter of the ventricular septal defect is less than the diameter of the aortic root. This must be done to successfully create the ventriculo-aortic tunnel. The measurement of left ventricular pressure is often difficult in these patients and cannot always be done by a retrograde approach because the aorta arises from the right ventricle. Approach to the left ventricle from an atrial septal defect or the ventricular septal defect may be successful.
Sridaromont et aL l in their review of double outlet right ventricle reported only I case in 62 with an obstructive ventricular septal defect. I wonder whether the true incidence of this complication might have been underestimated since very few left ventricular pressures were recorded. We have en-
622
April 1977
The American Journal of CARDIOLOGY
Volume 39
Somkid Sridaromont, MD Department of Pediatrics Pediatric Cardiologist Texas Tech School of Medicine Lubbock, Texas
of our study, I soon realized that we had no clear idea what the big picture was. Had the pathologic findings changed from year to year over the past decade? We had no idea. Then we gradually realized that no one else seemed to know either! The large literature on the pathology of congenital heart disease consisted either of anecdotal case reports or of selected series--"numerators" without "denominators." This is why there are no pathologic findings, of which we are aware, with which our data can be compared. In this sense, we hope that our paper will begin a new genre inthe literature concerning the pathology of congenital heart disease. Similar studies from other centers are needed before it will be possible to see our findings concerning the postoperative pathology of congenital heart disease in perspective. These are the major reasons why we attempted to stress the enormous importance of reporting data and results in a complete and unselected way. Control series and statistical analysis in scientific publications are now generally understood to be desirable. But the need for completeness in reporting data and results is still largely unappreciated. Consequently, it is often not possible to ascertain the overall resuits for a given type of congenital heart disease at a particular institution. Moreover, it usually is not possible to compare, in a meaningful way, the surgical results in a given anomaly at several different institutions. This is why we suggested that the reporting of data and results should be complete and unselected. We think that this is a principle of basic importance and we hope that authors, reviewers and editors will take note. Application of this simple principle will considerably improve both the scientific quality and the practical usefulness of the medical literature. Richard Van Praagh, MD, FACC Children's Hospital Medical Center Harvard Medical School Boston, Massachusetts
OBSTRUCTING VENTRICULAR SEPTAL DEFECT IN DOUBLE OUTLET RIGHT VENTRICLE
countered three patients with an obstructive ventricular septal defect 2,:~ in our series of approximately 60 cases of double outlet right ventricle. The diagnosis of obstructive ventricular septal defect has important surgical implications because it may be necessary to enlarge the defect to ensure satisfactory postoperative hemodynamics. Also, surgical techniques may have to vary according to the type of obstruction, whether muscular or fibrous. 3 Preoperative diagnosis of the two varieties may be obtained by selective angiocardiography. Maria Serratto, MD, FACC Department of Pediatric Cardiology Cook County Children's Hospital Chicago, Illinois References 1. Srldaromont S, Feldt RH, Ritter DG, el al: Double outlet right ventricle: hemodynamic and anatomic correlations. Am J Cardio138:85-94, 1976 2. Serratto M, Arevalo F, Goldman EJ, el al: Obstructive venlricular septal defect in double outlet right ventricle. Am J Cardiol 19:457-643, 1967 3. Pellegrlno PA, Eckner FA, Meier MA, el al: Double outlet right ventricle with f~romuscular obstruction to left ventricular outlet. J Cardiovasc Surg 14:253-260, 1973
REPLY
We have now observed an obstructing ventricular septal defect in 3 of a total of 72 patients with double outlet right ventricle with levocardia. Two of those with significant ventricular septal defect obstruction demonstrated by pressure measurement were not part of our previous report because full hemodynamic data were not available. We agree that the diagnosis of obstructive ventricular septal defect has important implications. It is necessary, under those circumstances, for the surgeon to enlarge the ventricular septal defect when the diameter of the ventricular septal defect is less than the diameter of the aortic root. This must be done to successfully create the ventriculo-aortic tunnel. The measurement of left ventricular pressure is often difficult in these patients and cannot always be done by a retrograde approach because the aorta arises from the right ventricle. Approach to the left ventricle from an atrial septal defect or the ventricular septal defect may be successful.
Sridaromont et aL l in their review of double outlet right ventricle reported only I case in 62 with an obstructive ventricular septal defect. I wonder whether the true incidence of this complication might have been underestimated since very few left ventricular pressures were recorded. We have en-
622
April 1977
The American Journal of CARDIOLOGY
Volume 39
Somkid Sridaromont, MD Department of Pediatrics Pediatric Cardiologist Texas Tech School of Medicine Lubbock, Texas