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Ebstein's anomaly
THE AJC 25 YEARS AGO
The AJC in August 1958
CAROLE A. WARNES, MB, BS, MRCP and WILLIAM C. ROBERTS, MD
Hemodynamics of Mitral Regurgitation (Julio C. Davila) This report, part of the symposium on mitral regurgitation (MR), describes hemodynamic features of MR and physiologic sequelae of surgical reduction of mitral anular size. Of 128 patients with significant MR, with or without mitral stenosis, all had right-sided cardiac catheterization, and 22 had left atria1 puncture. Of 58 patients operated on, 56 had hemodynamic studies performed at surgery, before and after either partial or complete elimination of MR. The left-sided pressure changes of MR were documented at operation before and after mitral valve manipulation. The hemodynamic derangements usually reverted to normal. The left ventricular (LV) end-diastolic pressure and total LV volume, however, sometimes failed to revert to normal, and often these patients died despite apparent diminution or elimination of MR by operation. The author emphasized that patients with long-standing severe MR may not be benefited by the operation because when LV dilatation, the major compensation of MR, is severe, correction of MR actually may aggravate myocardial dysfunction by abolishing the “escape exit” into the low pressure left atrium and thus may increase LV work. He concluded that operative candidates should be selected before irreversible damage to LV myocardium had occurred. This report introduced important concepts of surgical treatment of MR, and is a fine example of physiologic orientation of early surgeons of valvular heart disease. Modern Concepts of Hypertension and Its Treatment (Joseph H. Hafkenschlel, Edward Weiss, William Llkoff, John K. Clark, and Charles C. Wolferth) This 19-page article reports a conference on systemic hypertension (SH) at Lankenau Hospital, Philadelphia. SH was considered to have 3 major causes: hereditary, renal, and psychogenic. This latter concept was given considerable emphasis, and the “personality inside an individual” was considered etiologically important in most patients. A study of 130 patients with SH who were
followed up for >5 years was reported: Only 66% survived for 5 years. Adrenalectomy for refractory SH was still considered acceptable therapy, but the conference invoked considerable dispute regarding the necessity, if any, for medical treatment for less severe forms of SH. Weiss stated, “In the so-called malignant SH, it has been demonstrated that ganglionic blockers prolong the life of these patients. Beyond that I have not seen any adequate demonstration that the drug treatment of SH prolongs life or changes the course of the vascular disease.” Medical therapy consisted essentially of rauwolfia and hydralazine. The results of chlorothiazide, then a new drug, were considered impressive. Some clinicians, however, remained convinced that drug therapy was used too early and objected to the use of potent drugs in the treatment of early or mild SH. This conference reflects the paucity of scientific data then available on SH. No definition of SH was given. In the last 25 years, the importance of treating SH has become obvious, and treatment has become more vigorous and introduced at earlier stages. However, recent observations on mild SH treated with thiazide diuretic drugs suggest a reconsideration of this approach. Ebsteln’s Anomaly (Joseph 6. Vacca, Donald W. Bussman, and J. Gerard Mudd) This report describes 3 cases of Ebstein’s anomaly, and reviews the data on 105 cases previously reported on since Ebstein’s original description in 1866: 55 were diagnosed clinically and 53 at necropsy. The typical clinical, radiographic, and electrocardiographic findings were described, and the frequent association of atrial septal defect and rhythm abnormalities was noted. Cardiac catheterization was considered helpful and diagnostic, but was acknowledged to be hazardous and possibly dangerous in patients with this anomaly. Diagnosis of Ebstein’s anomaly now is obviously much easier because of echocardiography with or without use of the intracavitary electrode. Despite the improved diagnostic accuracy and more successful treatment of rhythm abnormalities, however, surgical treatment remains less than ideal, and the overall survival has probably improved only slightly in the last 25 years.
The AJC in August 1958
CAROLE A. WARNES, MB, BS, MRCP and WILLIAM C. ROBERTS, MD
Hemodynamics of Mitral Regurgitation (Julio C. Davila) This report, part of the symposium on mitral regurgitation (MR), describes hemodynamic features of MR and physiologic sequelae of surgical reduction of mitral anular size. Of 128 patients with significant MR, with or without mitral stenosis, all had right-sided cardiac catheterization, and 22 had left atria1 puncture. Of 58 patients operated on, 56 had hemodynamic studies performed at surgery, before and after either partial or complete elimination of MR. The left-sided pressure changes of MR were documented at operation before and after mitral valve manipulation. The hemodynamic derangements usually reverted to normal. The left ventricular (LV) end-diastolic pressure and total LV volume, however, sometimes failed to revert to normal, and often these patients died despite apparent diminution or elimination of MR by operation. The author emphasized that patients with long-standing severe MR may not be benefited by the operation because when LV dilatation, the major compensation of MR, is severe, correction of MR actually may aggravate myocardial dysfunction by abolishing the “escape exit” into the low pressure left atrium and thus may increase LV work. He concluded that operative candidates should be selected before irreversible damage to LV myocardium had occurred. This report introduced important concepts of surgical treatment of MR, and is a fine example of physiologic orientation of early surgeons of valvular heart disease. Modern Concepts of Hypertension and Its Treatment (Joseph H. Hafkenschlel, Edward Weiss, William Llkoff, John K. Clark, and Charles C. Wolferth) This 19-page article reports a conference on systemic hypertension (SH) at Lankenau Hospital, Philadelphia. SH was considered to have 3 major causes: hereditary, renal, and psychogenic. This latter concept was given considerable emphasis, and the “personality inside an individual” was considered etiologically important in most patients. A study of 130 patients with SH who were
followed up for >5 years was reported: Only 66% survived for 5 years. Adrenalectomy for refractory SH was still considered acceptable therapy, but the conference invoked considerable dispute regarding the necessity, if any, for medical treatment for less severe forms of SH. Weiss stated, “In the so-called malignant SH, it has been demonstrated that ganglionic blockers prolong the life of these patients. Beyond that I have not seen any adequate demonstration that the drug treatment of SH prolongs life or changes the course of the vascular disease.” Medical therapy consisted essentially of rauwolfia and hydralazine. The results of chlorothiazide, then a new drug, were considered impressive. Some clinicians, however, remained convinced that drug therapy was used too early and objected to the use of potent drugs in the treatment of early or mild SH. This conference reflects the paucity of scientific data then available on SH. No definition of SH was given. In the last 25 years, the importance of treating SH has become obvious, and treatment has become more vigorous and introduced at earlier stages. However, recent observations on mild SH treated with thiazide diuretic drugs suggest a reconsideration of this approach. Ebsteln’s Anomaly (Joseph 6. Vacca, Donald W. Bussman, and J. Gerard Mudd) This report describes 3 cases of Ebstein’s anomaly, and reviews the data on 105 cases previously reported on since Ebstein’s original description in 1866: 55 were diagnosed clinically and 53 at necropsy. The typical clinical, radiographic, and electrocardiographic findings were described, and the frequent association of atrial septal defect and rhythm abnormalities was noted. Cardiac catheterization was considered helpful and diagnostic, but was acknowledged to be hazardous and possibly dangerous in patients with this anomaly. Diagnosis of Ebstein’s anomaly now is obviously much easier because of echocardiography with or without use of the intracavitary electrode. Despite the improved diagnostic accuracy and more successful treatment of rhythm abnormalities, however, surgical treatment remains less than ideal, and the overall survival has probably improved only slightly in the last 25 years.