Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome.

OBJECTIVES. To determine whether right ventricular preservation is compromised in any anatomic subgroup of hypoplastic left heart syndrome, we assessed the coronary artery anatomy and myocardial histologic features of 151 postmortem specimens with hypoplastic left heart syndrome. BACKGROUND. Although previous studies have shown that coronary artery abnormalities are more prominent in the subgroup of patients who have a patent mitral valve and obstructed aortic valve, it has not been established that these abnormalities compromise right ventricular perfusion. METHODS. Eighty-nine specimens with a patent mitral valve and aortic atresia, 52 with mitral and aortic atresia and 10 with patent mitral and aortic valves were examined. Histologic sections of the right ventricle, left ventricle and coronary arteries were obtained from 64 study hearts and 5 control hearts. RESULTS. Gross coronary anomalies included coronary-cameral communications (n = 29), single left coronary artery (n = 2), single right coronary artery (n = 1) and tortuosity (n = 19). Coronary-cameral communications and tortuosity were significantly associated with the subgroup that had mitral hypoplasia and aortic atresia. Coronary artery wall thickness relative to lumen diameter was not different among the subgroups. No areas of coronary artery stenosis or interruption were discovered. Although endocardial fibroelastosis of the left ventricle was strongly associated with the mitral hypoplasia and aortic atresia group, the right ventricular histologic findings were similar in all subgroups and were not differentially affected by older age. CONCLUSIONS. Although the incidence of coronary abnormalities is greater in patients with mitral hypoplasia and aortic atresia, in this study there was no apparent difference in perfusion of the right ventricle among the anatomic subgroups of hearts with hypoplastic left heart syndrome.     

Mitral valve prolapse in patients with coronary artery disease. Echocardiographic-angiographic correlation.

Echocardiography was performed in 25 consecutive patients with angina pectoris and angiographically demonstrable coronary artery disease. Left ventricular echograms detected late or pansystolic mitral valve bowing suggesting of mitral valve proplapse in 6/25 (24%). Left ventricular angiography showed prolapse of the posterior mitral leaflet in 15/25 (60%), including 5 detected by echocardiography. Significant triple vessel coronary disease was present in 11 of 15 patients with prolapsed mitralvalve. In each of the latter a greater than 90 per cent obstructive lesion was noted in at least one coronary artery: right coronary artery, 9 subjects (82%); left circumflex coronary artery, 5 patients (33%); and left anterior descending coronary artery, 4 patients (27%). Of 15 subjects with angiographic evidence of mitral valve prolapse, 13 had left ventricular asynergy-inferior or inferoposterior in 8 subjects (62%) and anterior or anteroapical in 5 subjects (38%). Eleven subjects had vectorcardiographic evidence of transmural myocardial infarction-inferior or inferoposterior in 9 (82%) and anteroseptal in 2 (18%). A single subject with mitral valve prolapse had mild mitral regurgitation. It is concluded that: (1) coexisting prolapse of the posterior mitral valve leaflet and coronary artery disease is usually associated with triple vessel obstructive lesions, (2) severe right coronary disease, inferior left ventricular wall asynergy, and inferior myocardial infarction are important angiographic and vectorcardiographic correlates, and (3) echocardiography will detect such mitral valve prolapse in only one-third of affected cases.     

Mitral valve prolapse in coronary artery disease.

Mitral valve motion, left ventricular segmental contraction and severity of arterial stenosis were analyzed in 92 patients with coronary artery disease and 28 patients with "atypical chest pain" and normal coronary arterio-rams. Mitral valve motion was evaluated for the presence or absence of leaflet prolapse. Segmental contraction was evaluated by calculating the percent shortening of six chords of the left ventricle measured from right anterior oblique ventriculograms. The severity of disease in each coronary vessel (left anterior descending, left circumflex and right coronary) was graded on a scale of 1 (0 to 30 percent stenosis) to 5 (complete occlusion). Mitral valve prolapse was not suspected clinically but observed angiographically in 15 of 92 patients with coronary artery disease and in 5 of 28 patients with normal coronary arteriograms. In nine patients with coronary artery disease, the prolapse was restricted to the posterior leaflet, in five it was in both the anterior and the posterior leaflets and in one patient in the anterior leaflet only. Mitral regurgitation was noted in seven patients with coronary artery disease; it was mild in six and moderate in one. Among the patients with coronary artery disease, 12 of the 15 (80 percent) with mitral valve prolapse had left ventricular asynergy compared with 63 of the 77 (82 percent) without valve prolapse. The mean scores for severity of disease in the left anterior descending, circumflex and right coronary arteries were, respectively, 4.2, 2.5 and 3.2 in the patients with valve prolapse and 4.2, 2.2 and 3.5 in those without prolapse. In summary, there was no significant correlation between mitral valve prolapse and distribution of coronary arterial obstructions or abnormal patterns of left ventricular segmental contraction. There was a high frequency of mitral valve prolapse in patients with severe coronary artery disease and in those with normal coronary arteriograms and atypical chest pain.     

Exercise left ventricular performance in patients with mitral valve prolapse

Patients with mitral valve prolapse may, even in the absence of associated coronary artery disease or significant mitral regurgitation, have abnormality in exercise left ventricular function. The precise reason for this abnormality, which appears to be age and sex related, is not clear. Abnormal ejection fraction response to exercise cannot be predicted by the nature of symptoms, electrocardiographic changes, arrhythmias, or by extent and severity of mitral valve prolapse by echocardiography. Caution should therefore be exercised in diagnosing associated coronary artery disease based on the ejection fraction response to exercise per se or even on exercise-induced wall motion abnormality. Patients with prolapse, have reduced exercise tolerance, which has been ascribed to reduced left ventricular filling and smaller left ventricular end-diastolic volume in the upright position. Patients with mitral valve prolapse and associated coronary artery disease or significant mitral regurgitation often have, as expected, abnormal left ventricular function during exercise.     

Mitral valve prolapse due to coronary artery disease

Controversy exists concerning the etiologic role of coronary artery disease in the prolapsing mitral valve leaflet syndrome. A 35 year old man with progressive coronary artery disease is described. Auscultation before and after his first myocardial infarction revealed only a fourth heart sound; subsequent left ventricular cineangiography demonstrated normal anatomy and function of the mitral valve, despite extensive wall motion abnormalities. Six months later he experienced another myocardial infarction after which the typical mid-systolic click, late systolic murmur of mitral valve prolapse developed. A second left ventricular cineanglogram at this time revealed mid-systolic mitral valve prolapse and mitral regurgitation. This patient's course indicates that myocardial damage from coronary artery disease can cause mitral valve prolapse in patients without preexisting redundant mitral valve tissue.     

Anomalous coronary artery origin associated with bicuspid aortic valve in a patient with rheumatic mitral stenosis: a case report

A rare case of an anomalous left coronary artery arising from the right sinus of Valsalva associated with bicuspid aortic valve is presented. This case is unique because these congenital anomalies were associated with rheumatic mitral stenosis. This anomalous coronary origin was found at catheterization before balloon mitral valvuloplasty. The clinical significance of this finding is discussed.     

Cardiac function during rest and exercise in patients with mitral valve prolapse: Role of radionuclear angiocardiography

The clinical features of mitral valve prolapse syndrome and ischemic coronary disease overlap, making differentiation of the two conditions difficult. Furthermore, many patients have both conditions. This study assessed changes in ventricular function during rest and exercise in patients with mitral valve prolapse alone and in patients with prolapse and concomitant coronary artery disease. Twelve patients with angiographically documented mitral valve prolapse and normal coronary anatomy and 11 patients with normal coronary anatomy and no mitral valve prolapse had increased ejection fraction and demonstrated no wall motion abnormality during exercise. Changes in ventricular function during exercise in 11 additional patients with mitral valve prolapse demonstrated on echocardiography were similar to those in the group with mitral valve prolapse and normal coronary anatomy seen on angiography. In contrast, 6 of 11 patients with mitral valve prolapse and coronary arterial stenosis demonstrated on angiography had a decreased ejection fraction and exhibited wall motion abnormalities during exercise. These results suggest that mitral valve prolapse alone has no detrimental effect on ventricular function during rest and exercise and that exercise-induced abnormalities in ventricular function are related to the presence and severity of coronary artery disease and not to mitral valve prolapse.     

Mitral valve prolapse in short-term experimental coronary occlusion: a possible mechanism of ischemic mitral regurgitation

Experimental coronary occlusions were carried out in 12 closed-chest dogs to investigate the functional anatomic characteristics of the mitral valve complex during acute myocardial ischemia. Two-dimensional echocardiography was used to assess left ventricular function, the mitral valve complex, and left atrial size. Presence of mitral regurgitation was assessed by left ventricular contrast echocardiography. Thirty-seven coronary occlusions of up to 10 min in duration were carried out in proximal or distal locations in the left anterior descending and the left circumflex coronary arteries. Mitral regurgitation, which was mild in severity as judged by a small rise in pulmonary artery wedge pressures, was observed in 15 of 37 brief coronary occlusion experiments. Mitral valve prolapse was noted in all 15 experiments, as well as in four additional studies in which mitral regurgitation was not seen. The development of experimental mitral valve prolapse was explained by measurements that demonstrated a relative displacement of the papillary muscle tips toward the mitral orifice. We conclude that mitral valve prolapse is a common sequela of short-term coronary occlusion and is often associated with mild mitral regurgitation. Relative displacement of ischemic papillary muscles toward the mitral orifice appears to be a likely mechanism of acute ischemic mitral valve prolapse.     

Myocardial bridging confined to the right ventricular branch of the right coronary artery in a patient with severe pulmonary hypertension

Although myocardial bridges are mostly confined to the left anterior descending coronary artery, several cases of right coronary artery myocardial bridging have been reported in the literature. In the current case report, we present a 65-year-old female with a well-functioning mechanical mitral valve prosthesis, severe pulmonary hypertension and right ventricle wall motion abnormality in whom diagnostic angiography revealed myocardial bridging confined to the right ventricular branch of the right coronary artery.     

Coronary vessel anomaly: fistula between the right coronary artery and the left ventricle clinically presenting as aortic valve insufficiency (author's transl)]

Under more than 200 cases of coronary vessel anomalies only 33 are fistulas from the right or left coronary artery into the left ventricle. The case presented here of a fistula from the right coronary artery into the left ventricle featured the clinical picture of an aortic valve insufficiency. The histologic findings support the theory that these fistulas are embryological anomalies: transient communications between the ventricular trabeculas and the coronary vessels may be lined with endothelium and become then persisting as fistulas.     

Prolapse of the mitral valve: clinical spectrum and coronary arterial distribution

Coronary angiograms were reviewed in 31 patients with idiopathic prolapse of the posterior mitral leaflet. There were 19 males and 12 females, ranging in age from 33 to 69. The coronary artery which supplied the posterior descending branch was designated as dominant. There were 27 dominant right coronary arteries and 4 dominant left coronary arteries. Attention was paid to whether the origin of the vessel which courses in the posterior atrioventricular groove branch was from the right coronary artery or the left circumflex. In the dominant right coronary artery group, the arterioventricular groove branch arose from the right coronary artery alone in 6 and from the left circumflex alone in 1 patient, and in 20 patients, from both. In the dominant left coronary artery group, the atrioventricular groove branch arose from the left coronary artery in all 4 patients. The frequency of dominant right coronary artery and left coronary and the origin of the atrioventricular groove branch did not differ in the patients with prolapse of the mitral valve from a control group of 30 patients similarly analyzed. In all instances, the atrioventricular groove branch arose from either the right coronary artery ro the left circumflex, or both. In no case was the arterioventricular groove branch totally absent. The results of this investigation do not support the thesis, previously advanced by others, that prolapse of the mitral valve is related to absence of the left circumflex coronary artery, but indicate a normal range of variation in coronary arterial distribution.     

Anomalous right coronary artery from the main pulmonary artery in a patient with double-chambered right ventricle

We describe a rare case of double-chambered right ventricle (DCRV) in a 32-year-old female presenting to the echocardiography lab for evaluation of congenital heart disease. We identified a unique constellation of findings, including the DCRV, a perimembranous ventricular septal defect, aortic valve prolapse, patent foramen ovale, and an anomalous right coronary artery coming off the main pulmonary artery. To the best of our knowledge, this is the first reported case describing the association of an anomalous right coronary artery coming off the main pulmonary artery in a patient with DCRV.     

Mitral valve prolapse causing severe mitral regurgitation in a patient with absent right pulmonary artery

A 33-year-old man was investigated for dyspnea on exertion and the presence of a pansystolic murmur. Physical examination revealed dextrocardia confirmed by chest radiograph, which also showed oligemic right lung field. Subsequent cardiac catheterization revealed secundum atrial septal defect, persistent left sided superior vena cava, and severe mitral valve prolapse causing severe mitral regurgitation with pulmonary hypertension. The right pulmonary artery was absent. It is the first report of the association between severe mitral valve prolapse and absent right pulmonary artery.     

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.     

Coronary artery spasm and mitral valve prolapse

Ten patients, representing 1.34 per cent of those patients undergoing selective coronary arteriography, were found to have unequivocal evidence of coronary artery spasm. This involved the proximal right coronary artery in eight patients, the mid-left anterior descending branch in one, and the left main coronary artery in one. Eight of these 10 patients had otherwise normal coronary arteries.Of these 10 patients with coronary artery spasm, nine had evidence of mitral valve prolapse. This involved the posteromedial scallop in six patients; the anterolateral and posteromedial scallops in one; the middle and posteromedial scallops in one; and the anterolateral, middle, and posteromedial scallops in one.These data suggest an association between coronary artery spasm and mitral valve prolapse. Coronary artery spasm may thus be an important factor in the pathogenesis of the chest pain, arrhythmias, electrocardiographic abnormalities, and sudden death, that have already been described in some patients with mitral valve prolapse.     

Bicoronary-pulmonary fistulae with coexistent mitral valve prolapse: a case report and literature review of coronary-pulmonary fistula

Bicoronary-pulmonary artery fistulae are rare congenital coronaryartery fistulae. We report the case of a 57-year-old man whopresented with biventricular failure and angina pectoris. Atangiography the coronary arteries were normal, but bicoronary-pulmonaryfistulae were noted; severe mitral regurgitation secondary tomitral valve prolapse was also present. At right heart catheterizationthe calculated left to right shunt was 2.1. Fistula ligationand repair of both mitral and tricuspid valves was undertaken.The literature regarding coronary-pulmonary fistula is alsoreviewed.     

Right ventricular subendocardial infarction in a patient with pulmonary hypertension,right ventricular hypertrophy,and normal coronary arteries

Right ventricular infarction is usually associated with coronary artery disease and concomitant left ventricular infarction. Isolated right ventricular subendocardial necrosis was discovered at autopsy in a 52-year-old woman with pulmonary hypertension, right ventricular hypertrophy, and normal coronary arteries, who died with septicemia 41 days after mitral valve replacement. This represents the first well-documented report of isolated right ventricular subendocardial infarction associated with normal coronary arteries.     

Mitral valve prolapse and coronary artery disease. Clinical, hemodynamic, and angiographic correlations.

Among 95 patients with angina pectoris and angiographically documented coronary artery disease (CAD), prolapse of the scallops of the posterior leaflet of the mitral valve (PLMV) was noted in 30 patients. Left ventriculograms in the right anterior oblique (RAO) projection revealed isolated prolapse of the posteromedial commissural scallop (PMCS) in 12 patients and the anterolateral commissural scallop (ALCS) in two patients. Seven patients had prolapse of both PMCS and ALCS, three had prolapse of the PMCS and middle scallop (MS), and six had prolapse of all three scallops of the PLMV. Left ventricular dilatation with increase trabeculations was observed in 19 patients. Contractility determined in a quantitative fashion by segmental motion analysis was markedly impaired in 29 patients. None of the patients had angiographic evidence of mitral insufficiency. Left ventricular dysfunction was documented in 28 patients by either elevated left ventricular end-diastolic pressure (LVEDP), low cardiac index (CI) or decreased ejection fraction (EF). In two patients in whom left ventricular contractility improved after aortocoronary by pass, previously prolapsed scallops could not be identified in the postoperative ventriculogram. Prolapsed PLMV is a frequent angiographic finding in patients with angiographically observed CAD. Impaired contractility of the ventricular myocardium and papillary muscles, left ventricular dilatation, and hypertrophy appear to play a significant role in the pathogenesis of this abnormality through distortion of the directional axis of the papillary muscles, asynergic contraction of the related free wall of the left ventricle, and changes in the normal spatial alignment necessary for mitral valve closure. The syndrome of papillary muscle dysfunction in patients with coronary artery disease represents a wider clinical spectrom than previously described.     

Wolff-Parkinson-White syndrome and cardiopathies

Forty-nine cases of Wolff-Parkinson-White syndrome (WPW) were diagnosed out of 10 750 patients with cardiac disease (0.45 p. 100), 24 cases out of 3 761 congenital malformations and 25 cases in the 6 989 patients with acquired heart disease. Right ventricular pre-excitation was recorded in 31 cases; 13 in the lateral zone, 12 in the posterior paraseptal zone and 6 in the anterior paraseptal zone. Left ventricular pre-excitation was recorded in 18 cases: 8 in the lateral zone, 5 in the anterior paraseptal and 5 in the posterior paraseptal zones. WPW and congenital heart disease: Out of 20 cases of Ebstein's anomaly, 5 cases of WPW were observed: 4 right posterior and 1 right lateral pre-excitations. Out of 218 cases of hypertrophic obstructive cardiomyopathy, 7 cases of WPW were observed, 4 of which were congenital. Three cases of WPW were recorded in 699 patients with ventricular septal defects. Out of 1 348 cases of atrial septal defect, 5 cases of pre-excitation were recorded, including 3 right posterior pre-excitations associated with an ostium primum defect. Pre-excitation was also observed in isolated cases of corrected transposition of the great arteries, supravalvular aortic stenosis, aortic incompetence and patent ductus arteriosus. Pre-excitation and acquired heart disease: Five cases of pre-excitation were recorded out of 305 cases of dilated cardiomyopathy (1.62 p. 100). Eleven cases of pre-excitation were recorded in a total of 3 471 cases of valvular heart disease (0.31 p. 100): 9 in rheumatic valve disease and 2 in mitral valve prolapse. Nine cases of pre-excitation were observed in 2 850 cases of coronary artery disease. Intermittent Wolff-Parkinson-White syndrome: Ventricular pre-excitation masks the ECG changes of complete right bundle branch block in Ebstein's anomaly, complete left bundle branch block in aortic incompetence and dilated cardiomyopathy, and the in-complete right bundle branch block often seen in mitral valve prolapse. The characteristic appearances of WPW depend on the zone of pre-excitation. Right ventricular hypertrophy observed in ventricular septal defect with pulmonary stenosis and mitral stenosis may be masked by right lateral pre-excitation. Changes of inferior wall myocardial infarction may be masked by left anterior wall pre-excitation. On the other hand, the effects of WPW on left ventricular hypertrophy are variable, high amplitudes of the resultant forces seeming to depend on late and isolated activation of one of the left ventricular walls.     

Causes of sudden death in competitive athletes

Cardiovascular diseases responsible for sudden unexpected death in highly conditioned athletes are largely related to the age of the patient. In most young competitive athletes (less than 35 years of age) sudden death is due to congenital cardiovascular disease. Hypertrophic cardiomyopathy appears to be the most common cause of such deaths, accounting for about half of the sudden deaths in young athletes. Other cardiovascular abnormalities that appear to be less frequent but important causes of sudden death in young athletes include congenital coronary artery anomalies, ruptured aorta (due to cystic medial necrosis), idiopathic left ventricular hypertrophy and coronary artery atherosclerosis. Diseases that appear to be very uncommon causes of sudden death include myocarditis, mitral valve prolapse, aortic valve stenosis and sarcoidosis. Cardiovascular disease in young athletes is usually unsuspected during life, and most athletes who die suddenly have experienced no cardiac symptoms. In only about 25% of those competitive athletes who die suddenly is underlying cardiovascular disease detected or suspected before participation and rarely is the correct clinical diagnosis made. In contrast, in older athletes (greater than or equal to 35 years of age) sudden death is usually due to coronary artery disease, and rarely results from congenital heart disease.