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Ventricular Septal Defect and Aorticregurgitation

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Ventricular Septal Defect and Aorticregurgitation Thorax: first published as 10.1136/thx.24.3.257 on 1 May 1969. Downloaded from Thorax (1969), 24, 257. Ventricular septal defect and aortic regurgitation K. A. HALLIDIE-SMITH, E. G. J. OLSEN', C. M. OAKLEY2, J. F. GOODWIN, AND W. P. CLELAND From the Unit of Clinical Cardiology, the Unit of Cardiothoracic Surgery, and the Department of Morbid Anatomy, Royal Postgraduate Medical School, London W.12 We have reviewed our experience of 29 patients with the association of ventricular septal defect and aortic regurgitation, 27 of whom have been treated surgically. Our present approach to the surgical management is outlined and the embryology and pathological anatomy are reviewed. The most common cause of aortic regurgitation was a prolapsed aortic valve cusp, which occurred in 19 patients. Aneurysmal dilatation of an aortic sinus accounted for the regurgitation in three patients, and two patients had both abnormalities. In two patients no cause for the aortic regurgitation was found. Those patients with aortic cusp prolapse commonly had -large ventricular septal defects, of which nearly a third were supracristal. Because of cusp prolapse into the ven- tricular septal defect, and sometimes into the right ventricular outflow tract, the findings at cardiac catheterization often suggested erroneously that the ventricular septal defect was small and that infundibular obstruction was present. Characteristic angiographic features have been demonstrated, in particular the deformed and prolapsed aortic cusp. The various techniques of surgical correction which were used are described, the majority of patients having had a patch closure of the ven- tricular septal defect and repair of the aortic valve. Some residual aortic regurgitation was usual, but it was improved or minimized in 18 of the 23 survivors, and the more recent results have been particularly encouraging. Closure of the ventricular septal defect alone relieved heart failure in a http://thorax.bmj.com/ 3-year-old child. There were four operative deaths (15% mortality) in patients with severe aortic regurgitation, three of whom were in heart failure before operation. The medical course was commonly of slowly increasing severity of the aortic regurgitation with progression to heart failure. However, the individual course was unpredictable and three children developed heart failure within a year of the onset of their aortic regurgitation. Infective endocarditis occurred in a quarter of the patients and usually followed the development of the aortic regurgitation. Despite the difficulties of surgical management it is felt that the unpredictable prognosis, high operative risk once cardiac failure has supervened, and the hazard of infective endocarditis make early operation desirable. on September 29, 2021 by guest. Protected copyright. The association of ventricular septal defect and THE PATIENTS STUDIED aortic regurgitation was first described by Laubry and, Pezzi in 1921. Since then, although only Between 1958 and 1967 we investigated 29 patients approximately 150 cases have been reported in the with ventricular septal defect and aortic regurgita- literature, the development of aortic regurgitation tion, 27 of whom had been surgically corrected. has come to be recognized as one of the more largest so far reported. This common and the most serious of the complica- This is the series tions of ventricular septal defect. group represents 12% of our series of 229 surgi- This combination of defects has a poor medical cally treated ventricular septal defects. Since the prognosis, and so with improving surgical tech- majority of the patients are specially referred this niques total correction is being more widely advo- figure may give a falsely high percentage of inci- cated. Surgical correction is not always easy, and dence. success depends largely on a comprehensive know- Our patients consisted of 17 males and 12 ledge of the anatomy and function of the aortic females, between 2 and 37 years of age at the valve. time of investigation, and 3 and 38 years at the time of operation, when 15 patients were less than 1'On a nt ftom the British Heart Fondation 2Supported by the BrIth Heart Foundation 16 years. 257 Thorax: first published as 10.1136/thx.24.3.257 on 1 May 1969. Downloaded from 258 K. A. Hallidie-Smith, E. G. J. Olsen, C. M. Oakley, J. F. Goodwin, and W. P. Cleland HISTORY The early history of the adult patients stolic murmur. Sometimes the murmurs were was difficult to elicit. Nine of the 15 children thought to be truly continuous, the diastolic gave a history of failure to thrive in infancy; murmur following the systolic murmur without 13 of the total number of patients complained of intermission. The majority of patients had a third some limitation of effort tolerance, and six devel- heart sound and a short apical mid-diastolic oped signs of heart failure. murmur. In 20 of the 29 patients a heart murmur was The aortic regurgitation was assessed as mild heard in early infancy, and in a further eight it in 5, moderate in 13, and severe in 11 on the was first noted between the ages of 3 and 8 years, basis of the clinical findings, electrocardiogram, usually at a school medical examination. The and chest radiograph. remaining patient, a West Indian, was never The most difficult differential diagnosis of examined in childhood. ventricular septal defect with aortic regurgitation The initial murmur was known to have been was from a sinus of Valsalva fistula into the right solely systolic in 19 patients. The time, of appear- ventricle. Other differential diagnoses considered ance of the diastolic murmur was well documented in some patients, particularly early in our experi- in 11 children, and varied between 2 and 12 years. ence, were patent ductus arteriosus, aorto- In eight other children the exact time of appear- pulmonary window, coronary artery fistula, and ance of the diastolic murmur was uncertain, but isolated aortic valve disease. Three patients had all of them were between 2 and 13 years when had a previous thoracotomy elsewhere for a it was first noted. The remaining patients pre- supposed patent ductus arteriosus. sented in adult life with an established diastolic murmur. PHONOCARDIOGRAPHY Recordings wore carried Seven of our patients (24%) provided a clear out in all the patients. They confirmed that the history of subacute infective endocarditis, but in most common murmurs were as described clini- none was it certain that the infection preceded cally but illustrated the similarity of the murmurs the aortic regurgitation. The aortic regurgitation, in some patients to those found in sinus of Val- however, preceded the subacute infective endo- salva fistula, patent ductus arteriosus, and aortic carditis in three patients. valve disease (Fig. 1). The second sound was widely split in the presence of infundibular pul- http://thorax.bmj.com/ PHYSICAL SIGNS Nine of the 15 children were monary stenosis. undersized, particularly in regard to weight. Bulging of the chest, particularly of the left side, ELECTAOCARDIOGRAPHY All the records showed was some evidence of left ventricular hypertrophy, common. ranging from slight in 12 to severe in five. There In all patients the pulses were described as was unequivocal evidence of jerky or ill-sustained. In 17 patients the diastolic biventricular hyper- arterial blood trophy in the three children who had infundibular pressure was below 40 mm. Hg, pulmonary stenosis requiring resection. Two and in 15 patients the arterial pulse pressure was on September 29, 2021 by guest. Protected copyright. 60 mm. or patients had incomplete right bundle-branch block more. while three, all with severe aortic regurgitation, A left ventricular impulse was noted in all the showed evidence of left atrial hypertrophy. The patients, and some degree of right ventricular frontal plane axis varied from +300 to +70° in hypertrophy in 14. A systolic thrill at the left all but twio patients, who had left sternal edge was common. Both the systolic and axis deviation of -30° and - 40° respectively, which was un- diastolic murmurs were commonly described as related to the severity of the aortic regurgitation maximal at the lower left sternal edge, but the or to the pulmonary area was thought to be the site of site or size of the ventricular septal maximum intensity in a few. defect. Several patients showed progressive left Three patients with ventricular hypertrophy, coinciding with progres- right ventricular infundibular stenosis had loud sion of systolic murmurs in the pulmonary area. the aortic regurgitation (Fig. 2). The murmurs were variously described, the RADIOGRAPHY There was some left ventricular description varying not only between different enlargement in all patients, and the cardiothoracic observers but between different examinations by ratio was over 50% in all save two. Right-sided the same observer. enlargement was noted in 16 patients, including Most often it was thought that the systolic the six in heart failure and three with a systolic murmur filled systole, stopping at the second right ventricular outflow tract gradient of over sound and followed by a decrescendo early dia- 50 mm. Hg. Ten patients showed some left atrial Thorax: first published as 10.1136/thx.24.3.257 on 1 May 1969. Downloaded from Ventricular septal defect and aortic regurgitation 259 A.i V.S.D. + V.S.D. + HA A.1 A.1 D.S. Z.B. A.A..- !! A.A AHA 'i !.i H.F. H.F . H.F - L.F. - ....... H4ILIXIIII 11."-11' V.S.D. + A.1 Sinus of valsolva defect P.D.A. W.-F t M.O. D.W. PA H.S.F.SEP.A.~ ~ M _~~~~~~~~~~~~~~~~~~~~~~~~~~LSE~ ~ ~ H HH.F. 1- v ^ .| -I | FIG. 1. Phonocardiograins of three patients with ventricular septal defect and aortic regurgitation compared with those of rheumatic aortic regurgitation, sinus of Valsalva fistula from aorta to right ventricle, and patent ductus arteriosus http://thorax.bmj.com/ respectively.
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