Surgical experience in patients with Marfan's syndrome, ascending aortic aneurysm and aortic regurgitation.

Surgical treatment of cardiovascular complications in patients with Marfan's syndrome is usually recommended with apprehension since the systemic nature of the disease predisposes to early and late complications. To define the incidence of these complications, 30 patients were evaluated after surgical treatment of aortic insufficiency and ascending aortic aneurysm at the Texas Heart Institute. To provide a minimal follow-up period of 5 years, only patients operated upon during of before 1968 were included in this series. There were 9 female and 21 male patients aged 4 to 80 years (mean 44 years). Aortic insufficiency was treated by valvuloplasty in 3 patients and by aortic valve replacement in 27. Graft replacement of the ascending aorta was required in 23 patients, and the aneurysm was excised and the aorta repaired by direct anastomosis in 7. Two patients were lost to follow-up study; 12 of the remaining 28 (42.8 percent) lived 5 years or more. The hospital mortality rate was 20 percent (6 of 30); the causes of death included dissection or rupture of the aorta in three patients, congestive heart failure in two and pulmonary embolism in one. The 24 survivors lived from 5 weeks to 9 years. Follow-up data were available on 22 of these patients. Ten of these (45.4 percent) died of late complications. Seven died suddenly, four of these had redissection, one patient had occlusion of the right coronary artery, and two had ventricular fibrillation of no apparent cause. The remaining three died of noncardiac causes. Although the risk of ascending aortic and aortic valve surgery in patients with Marfan's syndrome is high, 42.8 percent of the patients in our series survived 5 years or more. We believe that surgery should be recommended for patients with Marfan's disease who have dissection of the aorta or severe aortic regurgitation, or both.     

Variant angina pectoris: Clinical and anatomic spectrum and results of coronary bypass surgery

Twenty patients are described with the variant angina syndrome (recurrent angina at rest with S-T segment elevations occurring only during pain and no evolution of infarction). In contrast to patients previously reported on, all but one had progressive unstable angina before hospitalization. Angina was frequently associated with arrhythmias, including ventricular fibrillation (2 Instances), ventricular tachycardia (4), frequent ventricular premature beats (5), atrioventrlcular block (4), sinus bradycardia (2), sinoatrial exit block (1) and supraventricular tachycardia (1). Seventeen patients had significant proximal stenosis of one or more coronary arteries with good distal vessels. Bypass surgery in 15 of these patients resulted in one noncardiac postoperative death, one perloperative Infarction and relief of pain in all 14 survivors. After a 17 month mean follow-up period (range 4 to 38 months), all survivors are pain-free. Three patients had no significant coronary disease; one of these became asymptomatic with medical therapy, one continues to have angina and one died suddenly. Patients with normal coronary arteries could not be distinguished clinically or by electrocardiogram from those with severe obstructive lesions. This experience suggests that all patients with the variant angina syndrome should be studied by coronary angiography, and that most patients with significant fixed coronary lesions will do well after coronary bypass surgery.     

Penicillin allergy in cancer patients manifesting as Kounis syndrome

Two cases of allergic angina and allergic myocardial infarction (Kounis syndrome) following penicillin administration are described. The patients suffered from lung and mandible neoplasms and had previously received several courses of antineoplastic therapy without any sequelae. One patient had normal coronary arteries (type I variant of the syndrome) and the other had coronary artery disease with previous myocardial infarction (type II variant of the syndrome). The allergic reaction following penicillin administration seemed to have triggered the development of an acute coronary artery spasm in the first patient and an acute myocardial infarction in the second. This report shows that susceptible individuals expressing a magnified mast cell degranulation effect may be more vulnerable to coronary artery spasm and plaque erosion or rupture.     

Radiation heart disease. Analysis of 16 young (aged 15 to 33 years) necropsy patients who received over 3,500 rads to the heart

Certain clinical and necropsy findings are described in 16 young (aged 15 to 33 years) patients who received greater than 3,500 rads to the heart five to 144 months before death. All 16 had some radiation-induced damage to the heart: 15 had thickened pericardia (five of whom had evidence of cardiac tamponade); eight had increased interstitial myocardial fibrosis, particularly in the right ventricle; 12 had fibrous thickening of the mural endocardium and 13 of the valvular endocardium. Except for valvular thickening, the changes were more frequent in the right side of the heart than in the left, presumably because of higher radiation doses to the anterior surface of the heart. In six of the 16 study patients and in one of 10 control subjects, one or more major epicardial coronary arteries were narrowed from 76 to 100 percent in cross-sectional area by atherosclerotic plaque; one patient had a healed myocardial infarct at necropsy and one died suddenly. In 10 patients and in the 10 control subjects, the four major epicardial coronary arteries were examined quantitatively: 6 percent of the 469 five millimeter segments of coronary artery from the patients were narrowed from 76 to 100 percent (controls = 0.2 percent, p = 0.06) and 22 percent were narrowed from 51 to 75 percent (controls = 12 percent). The proximal portion of the arteries in the patients had significantly more narrowing than the distal portions. The arterial plaques in the patients were largely composed of fibrous tissue; the media were frequently replaced by fibrous tissue, and the adventitia were often densely thickened by fibrous tissue. In five patients, there was focal thickening (with or without luminal narrowing) of the intramural coronary arteries. Thus, radiation to the heart may produce a wide spectrum of functional and anatomic changes but particularly damage to the pericardia and the underlying epicardial coronary arteries.     

The coronary arteries in Marfan's syndrome. A morphologic study.

The coronary arteries of five hearts of patients known to have had Marfan's syndrome were studied microscopically. In four of the five cases the extramural coronary arteries showed structural changes consisting of accumulations of acid mucopolysaccharides and a concomitant disruption of the connective tissue elements. These changes closely resembled the changes that frequently occur in the aorta and that are generally described as "cystic medial necrosis". They are considered to be an important expression of wall weakening. In one case a laceration, probably traumatic, of a coronary artery led to formation of an "incomplete dissecting aneurysm." "Cystic medial necrosis" was found once in the proximal segment of the sinus nodal artery and once in the musculoelastic layer of a coronary artery that reached the atrioventricular node from the ventricular septum by perforating the anulus fibrosus. However, in contrast to findings in a previous report, the intramural coronary arteries were otherwise unaffected. The clinical significance of these data is as yet unknown particularly since these alterations are intramural and initially do not cause narrowing of the lumen. Nevertheless, the possibility of coronary arterial involvement by the metabolic disorder of Marfan's syndrome should always be considered during clinical assessment of this lesion.     

Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery

There is disagreement concerning the optimal management of infants with congestive heart failure due to anomalous origin of the left coronary artery from the pulmonary artery. This report describes treatment in 23 such patients who had congestive heart failure before age 6 months. Eight patients had an operation in the 1st year of life and only two (25 percent) of these patients survived. Fifteen patients received initial medical treatment, and operation was delayed until an average age of 7.5 years; 13 (87 percent) of these patients survived. The two patients who survived early operation are still alive at an average age of 2.5 years. The 13 survivors in the medical group are still alive at an average age of 14.5 years. The age at onset and the severity of congestive heart failure were similar in the two groups. The frequency of the electrocardiographic pattern of myocardial infarction was similar in both groups. The radiographic cardiothoracic ratio was 0.74 in the surgical and 0.66 in the medical group. In 10 patients the left ventricular ejection fraction was less than 20 percent in the 1st year of life. In 7 of these 10 patients operation was performed before age 1 year and 2 (28 percent) survived; of the 3 patients treated medically, 1 (33 percent) survived.The outcome of surgical or medical treatment of the infant with anomalous origin of the left coronary artery from the pulmonary artery may be biased by patient selection. In the infants with depressed left ventricular function, the results of surgical and medical treatment are equally disappointing. Because coronary arterial surgery in the 1st year of life in infants with severely depressed myocardial function has an extremely high mortality rate (75 percent) and because many symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery can be treated medically and survive, we recommend delay of operative intervention until at least age 18 months.     

Myocardial infarction before age 36: Risk factor and arteriographic analysis

One-hundred twenty consecutive patients who were 35 years of age or younger underwent coronary arteriography after clinical myocardial infarction. Ninety-two percent were men. Four distinct subgroups were identified: Ninety-four patients (78 percent) had significant coronary artery disease (greater than 50 percent diameter narrowing of at least one major coronary artery), 20 (17 percent) had normal coronary arteries, 5 (4 percent) had major coronary arterial anomalies and 1 patient had coronary arteritis. Of the patients with obstructive coronary disease, risk factors were smoking in 89 percent, positive family history of coronary artery disease in 48 percent, hypertension in 21 percent and a history of lipid abnormality in 20 percent. Risk factors were distinctly less frequent in the groups without coronary atherosclerosis. In the group with coronary artery disease, the prevalence rate of one, two and three vessel disease was 32, 26 and 42 percent, respectively. Coronary arterial anomalies included anomalous origin of the left coronary artery from the pulmonary artery (three patients) and single right and single left coronary artery (one patient each).It is concluded that myocardial infarction before age 36 is a disease of men who smoke and who often have a family history of premature coronary artery disease. Twenty-two percent of patients will have normal coronary arteries, coronary arterial anomalies or coronary vasculitis. Coronary arteriography should be considered for patients who sustain a myocardial infarction before age 36 for purposes of diagnosis, management and prognosis.     

Assessment of plexectomy in the treatment of severe coronary spasm in patients with normal coronary arteries

Surgical denervation of the heart by plexectomy was performed in 3 patients with variant angina, documented coronary spasm, and normal findings on coronary angiography. In all cases, spasm had already been responsible, preoperatively, for myocardial infarction and recurred thereafter in another territory despite medical therapy with a combination of nitrates and calcium antagonists. Plexectomy was performed using a standardized technique. The effectiveness of surgical suppression of cardiac autonomic innervation was confirmed postoperatively by pharmacologic tests. In 2 patients inferior myocardial infarction developed in the early postoperative period; in the third patient, coronary spasm recurred 3 weeks after plexectomy. Thus plexectomy, despite an adequate suppression of autonomic innervation, was ineffective in all cases and may even have been harmful in 2 patients. These data contradict the good results obtained by plexectomy associated with aortocoronary bypass in patients with variant angina and fixed stenotic coronary arteries. This discrepancy may be accounted for by a different pathophysiologic mechanism of vasospasm in normal coronary arteries and in diseased arteries at the site of the atheromatous stenosis. Thus, plexectomy should not be considered in the treatment of vasospasm involving normal coronary arteries, even if medical therapy fails to achieve satisfactory control of variant anginal attacks.     

Vascular complications of thorium dioxide.

Vascular complications occurring late after exposure to thorium dioxide (thorotrast) are described in two patients. One patient had both cerebral andmyocardial infarcts and died at age 25 years. Necropsy disclosed both adventitial and intimal fibrosis of the left carotid artery and greater than 75 per cent cross-sectional area luminal narrowing of both the left main and left anterior descending coronary arteries. The other patient, a 33 year old man, had no cerebral symptoms in life but total obstruction of the right carotid artery secondary to a thorotrastoma was found at necropsy. Vascular complications due to thorotrast appear to represent consequences of chronic alpha irradiation.     

Thallium-201 myocardial perfusion studies in patients with the mitral valve prolapse syndrome

To determine whether regional myocardial ischemia plays a role in patients with the mitral valve prolapse syndrome, we examined myocardial perfusion with exercise stress testing and thallium-201 myocardial scintigraphy. Twelve patients were studied, 11 women and one man aged 18 to 56 years, mean age 30 years. In all patients, mitral valve prolapse was documented by echocardiography or phonocardiography. Patients over 35 years of age underwent cardiac catheterization. Electrocardograms disclosed abnormalities during maximal exercise in eight of the 12 patients. In two patients, angina developed during exercise. Thallium-201 (²⁰¹TI) scintigrams were normal in the 11 patients with presumed or documented normal coronary arteries. One patient, in whom an apical defect was demonstrated on scintigraphy, had significant disease of the left main and left anterior descending coronary artery. Repeat testing after successful aortocoronary bypass grafting revealed improved exercise capacity and a normal ²⁰¹TI myocardial scintigram. The data indicate that patients with mitral valve prolapse alone do not have regional myocardial ischemia and that the presence of a defect on ²⁰¹TI myocardial scintigraphy following maximal stress testing would suggest the existence of concomitant coronary artery disease.     

Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries

Clinical and morphologic features of transmural myocardial infarction (associated with insignificant or absent atherosclerosis of the extramural coronary arteries) are described in seven patients with hypertrophic cardiomyopathy. Marked chronic congestive heart failure associated with supraventricular arrhythmias occurred in six of the seven patients, each of whom had no or mild left ventricular outflow tract obstruction under basal conditions. No patient had typical angina pectoris, and only one patient had clinically evident acute myocardial infarction. Infarction may have caused cardiac arrest in one other patient, but was “silent” in the remaining five patients.

At necropsy, six of the seven patients had extensive myocardial scarring involving the ventricular septum, left ventricular free wall and one or both left ventricular papillary muscles; in four patients portions of the right ventricular wall were also scarred. Six patients had dilated ventricular cavities, including two who were known to have nondilated ventricular cavities earlier in their clinical course.

It is concluded that transmural myocardial infarction in the absence of significant coronary atherosclerosis is a not uncommon finding (prevalence rate 15 percent) in a population of patients who had died from hypertrophic cardiomyopathy. Although transmural infarction is possibly a secondary event, it more likely contributes causally to the clinical deterioration of some patients with hypertrophic cardiomyopathy, leading to ventricular dilatation and progressive fatal cardiac failure.     

Large multiple coronary artery aneurysm in adult patients: a report on three patients and a review of the literature

Three cases of large multiple coronary aneurysms, situated on both right and left coronary arteries, were seen in three middle-aged adult patients. These patients were hospitalized for myocardial infarction in two cases and for angina pectoris in the third case. On the coronary angiogram, the coronary lesions were quite unusual as there were multiple voluminous aneurysms on both coronary arteries without evidence of atherosclerotic lesions of the remainder of the coronary tree. These lesions did not seem to be congenital or atherosclerotic, and it was postulated that these lesions might have been the sequelae of a mucocutaneous lymph node syndrome although no previous history of this condition could be found in these three patients.     

Coronary artery disease in young patients: arteriographic and clinical review of 40 cases aged 35 and under

Coronary arteriography was performed in 60 patients aged 35 or less with suggested coronary artery disease (CAD). Twenty patients (Group 1) had normal coronary arteries and 40 patients (Group 2) had one or more obstructive lesions. The left anterior descending artery was commonly involved followed by the right coronary and left circumflex arteries. The right coronary artery was most commonly completely obstructed. Single-vessel disease (50 per cent or greater obstruction) was found in 60 per cent of the patients, an incidence that is considerably higher than in studies of older patients. A total of 1.6 diseased vessels per patient was present. A hyperlipoproteinemia (HLP) was found in 68 per cent of Group 2 patients. Patients in Group 2 with an HLP had significantly more CAD than Group 2 patients with normal lipoproteins. The incidence of the following clinical features were not significantly different in Groups 1 and 2: typical angina, atypical angina, positive family history, smoking, hypertension, obesity, abnormal electrocardiogram, positive treadmill test, HLP, and diabetes mellitus. A fourth heart sound and a history of a myocardial infarction were significantly common in Group 2. Since almost all of the previously reported cases of myocardial infarction with normal coronary arteries have occurred in young patients, history of a myocardial infarction does not assure the presence of obstructive coronary artery lesions. It is suggested that coronary arteriography is a justifiable procedure in a young patient who presents with a clinical picture that is either compatible with or cannot be clearly distinguished from CAD.     

Multivessel coronary spasm in patients with variant angina: a study with intracoronary injection of acetylcholine

Multivessel coronary spasm has been described but its incidence in patients with variant angina still remains unclear. Thirty-three patients with variant angina were studied during coronary angiographic examination with selective intracoronary injection of acetylcholine (ACh). In all but three patients, the location of ischemia during attack was determined by the electrocardiographic findings, by exercise 201Tl myocardial scintigraphy, and by two-dimensional echocardiography during a hyperventilation test, and the coronary artery (or arteries) responsible for the attack was predicted before the study. ACh induced spasm of at least one coronary artery in all but one patient. ACh induced spasm of both the left and right coronary arteries (i.e., multivessel coronary spasm) in 24 patients: in two of the four patients who were predicted to have spasm of the left coronary artery, in six of the 11 predicted to have spasm of the right coronary artery, in 13 of the 15 predicted to have spasm of both the left and right coronary arteries, and in three of the three in whom coronary artery responsible for attack had not been predicted. This ACh-induced spasm of the left and right coronary arteries occurred separately and no patients showed hemodynamic instability during attack. In one patient in whom multivessel coronary spasm had been predicted and ACh failed to induice coronary spasm, ergonovine maleate (0.2 mg) induced spasm of both the left and right coronary arteries simultaneously, resulting in severe prolonged hypotension. Nineteen of the 25 patients in whom multivessel coronary spasm was documented showed angiographically normal or nearly normal coronary arteries after administration of nitroglycerin.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fibromuscular dysplasia of the coronary and renal arteries?

Fibromuscular dysplasia (FMD) involving the coronary arteries has been described pathologically as a cause of myocardial infarction but has not been described antemortem. Unlike renal artery FMD, its clinical manifestations remain poorly characterized. We describe demographic, clinical, and coronary angiographic characteristics of seven women with acute coronary syndromes and unusual coronary anatomy who also had renal artery FMD. All subjects were female caucasians, age 42-56, who presented with prolonged chest pain and positive troponin tests. Two were smokers, two had hypertension, and one had hypercholesterolemia. None was diabetic. There were distinctive angiographic features common to all seven patients. The left anterior descending artery was involved in six, the right posterior descending artery in one. In each case, the proximal vessel appeared normal but in the middle or distal segment there was a well-demarcated abrupt transition to diffuse obliterative disease. In six of the cases, this continued distally for the remainder of the epicardial vessel. In no case was revascularization feasible. Unlike severe diffuse atherosclerotic disease, all other coronary segments were angiographically normal. Ventricular dysfunction, if present, was mild. All seven patients had typical angiographic features of renal FMD, three bilaterally. We have observed a characteristic pattern of well-demarcated obliterative coronary artery disease associated with FMD of the renal arteries. All cases presented as acute coronary syndromes in patients at relatively low risk of coronary artery disease. We propose that these appearances in the epicardial arteries, previously undescribed ante-mortem represent coronary artery fibromuscular dysplasia.     

Dynamic Systolic Compression of the Left Anterior Descending Coronary Artery as the First Clue of Postinfarction Left Ventricular Pseudoaneurysm

Left ventricular pseudoaneurysm and left ventricular free wall rupture are rare but life-threatening complications of acute myocardial infarction. Dynamic systolic compression of the coronary arteries is almost always due to myocardial bridging. However, it has rarely been described in patients with ventricular aneurysms and pseudoaneurysms. We present a case of a patient with recent myocardial infarction who presented with recurrence of chest pain and coronary angiogram showing patent vessels however with systolic compression of the distal left anterior descending coronary artery as a first clue to postinfarct left ventricular pseudoaneurysm.     

Coronary heart disease after mediastinal irradiation for Hodgkin's disease.

Attention is called to the development of coronary heart disease in two patients several years after they received mediastinal irradiation for Hodgkin's disease. One patient, a 33 year old man, died suddenly eight years after irradiation; necropsy disclosed marked narrowing of all three major coronary arteries. In addition to severe intima fibrous thickening, there also was considerable adventitial scarring of the coronary arteries. This type of coronary sclerosis is different from that seen in the usual patient with coronary heart disease. The second patient, a 42 year old man, had an acute myocardial infarction on two occasions, the first six years after mediastinal irradiation. Observations in previously described patients with coronary heart disease almost surely induced by therapeutic irradiation for Hodgkin's disease are reviewed.     

Angina pectoris, myocardial infarction and sudden cardiac death with normal coronary arteries: a clinicopathologic study of 9 patients with progressive systemic sclerosis

The syndrome of angina pectoris or acute myocardial infarction without obstructive coronary artery disease has been the subject of much interest. We studied nine autopsied patients with progressive systemic sclerosis and evidence of ischemic heart disease but morphologically normal coronary arteries. Three patients had angina pectoris and three others chest pains of unknown etiology, six had ventricular arrhythmias, four had clinically suspected acute myocardial infarction, and eight had sudden cardiac death. At autopsy extensive focal myocardial necrosis was present in seven patients and myocardial scarring in all nine, but all patients had widely patent intramural and extramural coronary arteries. The finding of contraction band myocardial necrosis in seven of the eight patients who experienced sudden death suggests that the myocardial damage was a consequence of reperfusion of focally nonperfused myocardium, and thus due to a myocardial Raynaud's phenomenon. Patients with PSS may provide a model of spasm of intramyocardial vessels causing angina pectoris or myocardial infarction with morphologically normal coronary arteries.     

Prolonged and decremental antograde conduction properties in right anterior accessory connections: Wide QRS antidromic tachycardia of left bundle branch block pattern without Wolff-Parkinson-White configuration in sinus rhythm

Four patients are described who had long conduction times and decremental conduction through right anterior accessory connections. None had Wolff-Parkinson-White syndrome on their ECG. Each had recurrent sustained wide QRS tachycardia due to antegrade conduction through the accessory connection. Three patients underwent epicardial mapping and successful surgical division of their accessory connection. Two of the three had a second accessory connection that was also divided surgically. Each of the three are free of tachycardial without medication. It is postulated that these accessory connections represent the remnants of anterior atrioventricular ring tissue described by Anderson.     

"Ischemia at a distance" during intermittent coronary artery occlusion: a coronary anatomic explanation

The mechanism of electrocardiographic ST segment changes during acute coronary occlusion was evaluated in 28 consecutive patients with single vessel coronary artery disease undergoing coronary angioplasty. Patients were continuously monitored with a six lead electrocardiogram. Twenty-three patients showed ST changes in the primary zone of occlusion, and 13 of these had additional ST changes in a remote zone. Ten of these 13 had unusually extensive arteries supplying the remote zone. The balloon occluded two adjacent normal arteries in two patients, and no coronary anatomic explanation was evident in one patient. Ten patients with striking primary zone ST changes showed no remote change. Seven had nonextensive primary zone arteries, and three others had abundant collateral vessels. Five patients showed no electrocardiographic changes in primary or remote zones. Four had collateral vessels, and one had left ventricular hypertrophy on the baseline electrocardiogram. It was concluded that remote electrocardiographic changes are probably due to occlusion of unusually extensive coronary arteries and are not simply reciprocal.