ALCAPA in an adult with preserved left ventricular function

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is the most common congenital coronary artery defect. Left uncorrected, nearly 90% of patients will die within one year. Without surgical repair, surviving patients are subjected to risks of myocardial ischemia with global cardiomyopathy, chronic mitral regurgitation, and sudden death. We report a case of ALCAPA in a 23-year-old female with completely preserved left ventricular function. This article reviews the mechanism of this unusual presentation as well as the therapeutic options for adults presenting with ALCAPA.     

Reimplantation of the left coronary artery with aortic intramural course in an ALCAPA patient

We present a case of an anomalous origin of the left coronary artery from the pulmonary artery, with a proximal intramural course in the aortic wall. We describe the technique of its reimplantation at the site of the transverse aortotomy, using autologous pericardial patch enlargement of the newly created coronary ostium.     

Congenital left ventricular diverticulum repair in an adult with dual patch technique

Rupture of a congenital left ventricular diverticulum (CLVD), a rare anatomical anomaly, is a catastrophic event, with potential fatal consequences. Repair techniques documented in the literature include primary closure and single patch closure. We describe a case of a 57-year-old woman with symptomatic anterolateral CLVD. Our approach involves a linear incision through the epicardial surface of the diverticulum with exclusion of the cavity, and restoration of normal ventricular geometry via a two-patch technique.     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.     

Early assessment of rest and exercise left ventricular function following coronary artery surgery

Radionuclide assessment of rest and exercise left ventricular function was performed in 14 patients before, eight days after, and three months after coronary artery bypass grafting (CABG). Resting function was unaltered after operation, although mild increases in heart rate and end-diastolic volume were observed on the eighth postoperative day. In contrast, exercise function was significantly improved at both postoperative time periods. Exercise ejection fraction was 0.54 +/- 0.10 before operation, 0.73 +/- 0.12 at eight days, and 0.64 +/- 0.13 at three months. Before CABG, the exercise-induced increase in stroke volume was achieved by an increase in end-diastolic volume, whereas eight days after CABG this increase was achieved by an increase in contractility (systolic blood pressure/end-systolic volume). By three months, both contractility and end-diastolic volume increased with exercise. Thus, improvement in left ventricular function during exercise can be documented as early as eight days after coronary revascularization. This change may be less pronounced after three months of convalescence, but considerable improvement in ventricular function persists compared to preoperative assessment.     

Reverse remodeling and improved regional function after repair of left ventricular aneurysm

BACKGROUND: Changes in regional left ventricular mechanics after anteroapical aneurysm repair in human subjects can be studied noninvasively by means of magnetic resonance tagging. We hypothesized that left ventricular intramyocardial function would improve throughout the left ventricle after repair. METHODS: We studied 6 male patients with a left ventricular anteroapical aneurysm (mean age +/- SD, 63 +/- 5 years) using magnetic resonance tagging 3 +/- 1 weeks before and 6 +/- 1 weeks after aneurysm repair, coronary artery bypass grafting, and mitral valve repair (n = 2). Breath-hold tagged imaging spanned the left ventricle in the short axis from apex to base. Left ventricular mass, end-diastolic and end-systolic volume, and ejection fraction were measured. Two-dimensional strain analysis was applied; averaged for the apical, middle, and basal left ventricle and the whole left ventricle; and expressed as greatest lengthening (similar to wall thickening), greatest shortening, and angular deviation of the lengthening strain from the radial direction. RESULTS: After aneurysm repair, left ventricular mass decreased from 373 +/- 27 to 333 +/- 25 g (P <.05), end-diastolic volume from 212 +/- 22 to 168 +/- 18 mL (P <.005), and end-systolic volume from 188 +/- 26 to 113 +/- 18 mL (P <.005); ejection fraction improved from 13% +/- 4% to 23% +/- 4% (P <.005). For the whole left ventricle, lengthening strain increased from before to after the operation (8% +/- 1% to 10% +/- 1%, P <.01). Most of the improved lengthening occurred at the middle left ventricle (8% +/- 1% to 11% +/- 1%, P <.01), in the base (8% +/- 1% to 10% +/- 1%, P <.05), and in the inferior wall (9% +/- 1% to 12% +/- 1%, P <.05). Lengthening tended to become more radially oriented, decreasing from 31 degrees +/- 3 degrees to 27 degrees +/- 3 degrees (P =.10). Shortening strain did not change (10% +/- 1% to 11% +/- 1%, P = not significant). CONCLUSIONS: Left ventricular aneurysm repair is associated with reverse remodeling and an improvement in the extent and orientation of intramyocardial function, especially at the middle and basal left ventricle and inferior wall.     

Chronic chymase inhibition preserves cardiac function after left ventricular repair in rats.

OBJECTIVE: Although left ventricular repair (LVR) has been widely performed, the initial improvement of LV function does not last because of LV remodeling. Recent studies have demonstrated that chymase, a local enzyme in the heart, promotes angiotensin II formation as well as activation of transforming growth factor (TGF)-beta, both of which facilitate myocardial fibrosis. Therefore, chymase blockade may play an important role in the prevention of cardiac remodeling after LVR. In this study, the effects of chronic chymase inhibition (Chy-I) after LVR were evaluated in a rat LV aneurysm model. METHODS: Rats that developed LV aneurysms 4 weeks after coronary artery ligation underwent LVR by plicating the LV aneurysm, and were randomized into two groups, the LVR group and the LVR + Chy-I group that received an oral chymase inhibitor (10 mg/kg/day) for 4 weeks. RESULTS: Echocardiography revealed better LV function in the LVR + Chy-I group than in the LVR group at 4 weeks. Four weeks after LVR, LV end-diastolic pressure and the time constant of LV isovolumic pressure decay, were significantly lower in the LVR+Chy-I group. The end-systolic pressure-volume relationship was higher in the LVR+Chy-I group. In the LVR+Chy-I group, mRNA expressions of TGF-beta1 and BNP significantly decreased in the LV myocardium. Histology showed reduced interstitial fibrosis in the LVR+Chy-I group. CONCLUSIONS: Chronic chymase inhibition prevented myocardial fibrosis and preserved cardiac function after LVR. A chymase inhibition could be an important strategy for management after LV repair surgery.     

Evaluation of left ventricular function in coronary bypass surgery

OBJECTIVES: To evaluate non-invasive indexes measuring systolic and diastolic ventricular function. Eleven coronary artery bypass grafting (CABG) patients were investigated in order to assess the ability of preoperative ejection fraction (EF) and end diastolic pressure (EDP) to predict left ventricular function determined non-invasively at surgery. DESIGN: End-systolic elastance (Ees) was assessed perioperatively using transoesophageal echocardiographic area estimation and arterial pressure monitoring during preload variations (caval balloon). Diastolic function was evaluated using three different echo/Doppler indexes. RESULTS: EF correlated positively to Ees (r = 0.69, p = 0.03). No correlations were found between EDP and the perioperative diastolic indexes. Ees fell from pre-bypass to post-bypass (from 9.0 +/- 2.7 to 4.7 +/- 1.7 mmHg/cm2, mean +/- SD, p < 0.001), but no alterations in diastolic parameters occurred. CONCLUSIONS: A positive correlation was found between preoperative EF and Ees at surgery. The semi-invasive Ees detected a systolic "stunning" after cardiopulmonary bypass and is promising as a surveillance tool for left ventricular perioperative function and treatment. No correlations between preoperative EDP and non-invasive diastolic indexes were found, and assessment of perioperative diastolic function needs further refinement.     

Outcome of geometric endoventricular repair in impaired left ventricular function

BACKGROUND: Traditionally, repair of left ventricular aneurysms has been limited to patients with large localized ventricular aneurysms. Repair of dyskinetic segments in the setting of poor left ventricular function is still contentious. METHODS: Forty patients underwent geometric endoventricular repair, a new technique of ventricular aneurysm repair, over a 2-year period. Two groups of patients undergoing coronary artery bypass grafting (CABG) for left ventricular dysfunction in the same time period were reviewed. Group 1 comprised 23 consecutive patients who underwent geometric endo-ventricular repair along with CABGs, whereas group II consisted of 22 patients who underwent CABG alone. RESULTS: The early mortality was 9.1% in group I (1 cardiac, 1 noncardiac) and 0 in group II (NS). New York Heart Association class was remarkably improved from 3.4 to 1.4 (p < 0.05) in group I and to a lesser extent in group II (3.7+/-0.5 versus 2.3+/-0.5). Diastolic dimension of left ventricle was significantly reduced from 5.6 cm to 4.4 cm (p < 0.05) in group I and virtually unchanged in group II. There was one late death in each of the groups. CONCLUSIONS: This technique of geometric left ventricular aneurysm repair is useful in patients with dyskinetic segments and may help in reducing cardiac size.     

Beating heart ischemic mitral valve repair and coronary revascularization in patients with impaired left ventricular function

OBJECTIVE: The aim of this study is to evaluate in a cohort of patients with impaired left ventricular (LV) function and ischemic mitral valve regurgitation (MVR), the effects of on-pump/beating heart versus conventional surgery in terms of postoperative mortality and morbidity and LV function improvement. MATERIALS AND METHODS: Between January 1993 and February 2001, 91 patients with LVEF between 17% and 35% and chronic ischemic MVR (grade III-IV), underwent MV repair in concomitance with coronary artery bypass grafting (CABG) Sixty-one patients (Group I) underwent cardiac surgery with cardioplegic arrest, and 30 patients (Group II) underwent beating heart combined surgery. Aortic valve insufficiency was considered a contraindication for the on-pump/beating heart procedure. Mean age in Group I was 64.4 +/- 7 years and in Group II, 65 +/- 6 years (p = 0.69). RESULTS: The in-hospital mortality in Group I was 8 (13%) patients versus 2 (7%) patients in Group II (p > 0.1). The cardiopulmonary bypass (CPB) time was significantly higher in Group I (p < 0.001). In Groups I and II, respectively (p > 0.1), 2.5 +/- 1 and 2.7 +/- 0.8 grafts per patient were employed. Perioperative complications were identified in 37 (60.7%) patients in Group I versus 10 (33%) patients in Group II (p = 0.025). Prolonged inotropic support of greater than 24 hours was needed in 48 (78.7%) patients (Group I) versus 15 (50%) patients (Group II) (p = 0.008). Postoperative IABP and low cardiac output incidence were significantly higher in Group I, p = 0.03 and p = 0.027, respectively. Postoperative bleeding greater than 1000 mL was identified in 24 patients (39.4%) in Group I versus 5 (16.7%) in Group II (p = 0.033). Renal dysfunction incidence was 65.6% (40 patients) in Group I versus 36.7% (11 patients) in Group II (p = 0.013). The echocardiographic examination within six postoperative months revealed a significant improvement of MV regurgitation fraction, LV function, and reduced dimensions in both groups. The postoperative RF was significantly lower in Group II patients 12 +/- 6 (%) versus 16 +/- 5.6 (%) in Group I (p = 0.001). The 1, 2, and 3 years actuarial survival including all deaths was 91.3%, 84.2%, and 70% in Group I and 93.3%, 87.1%, and 75% in Group II (p = ns). NYHA FC improved significantly in all patients from both groups. CONCLUSION: We conclude that patients with impaired LV function and ischemic MVR may undergo combined surgery with acceptable mortality and morbidity. The on/pump beating heart MV repair simultaneous to CABG offers an acceptable postoperative outcome in selected patients.     

Differences in the recovery of right and left ventricular function after ischaemic arrest and cardioplegia

Differential susceptibility of right (RV) and left (LV) ventricles to plain ischaemia and the contribution of cardioplegia in reducing the ischaemic damage was evaluated in a sheep model of cardiopulmonary bypass. Sheep (n = 16) were equally divided for the two protocols of the study. Each sheep served as its own control. RV and LV functions were studied and compared by plotting ventricular function curves (Starling and Sarnoff). The mathematical centre of mass (COM) for each curve was computed by a polynomial regression program. The COM values of the contractility indices [stroke volume (SV), stroke work (SW)] represented ventricular heterometric reserve. RV and LV heterometric reserve was significantly (P less than 0.01) reduced after 1 h plain ischaemia, and quantitatively this change was more for RV than for LV (P less than 0.05). As compared to LV, a significantly greater reduction in RVSW was brought about by a significantly (P less than 0.01) greater reduction in RVSV, because there were no significant differences between the post-ischaemic recovery in the RV and LV afterloads. Cardioplegic protection nearly normalized filling pressure and contractility (SV, SW) of both ventricles, but major differences were observed in the recovery of compliance. Recovery in the RV compliance exceeded the control values, and quantitatively the recovery in RVSW was slightly more than in LVSW. The results of this study indicate that the RV suffers more damage from plain ischaemic arrest and hence benefits more from the cardioplegic protection.     

Early and midterm outcome after off-pump coronary artery bypass grafting in patients with poor left ventricular function compared with patients with normal function

OBJECTIVE: As there are few reports on the clinical results of off-pump coronary artery bypass (OPCAB) in patients with poor left ventricular (LV) function, the safety and efficacy of OPCAB surgery in such patients remains unclear. METHODS: From January 2002 to May 2007, a total of 519 consecutive patients underwent isolated myocardial revascularization via OPCAB. We compared the early and midterm results of 79 patients with poor LV function [ejection fraction (EF) < 40%] with those of 319 patients with normal LV function (EF >or= 50%). Follow-up was completed in 96.2% of the patients. RESULTS: There were no significant intergroup differences in the number of grafts per patient or the rate of achievement of complete revascularization. No patient in either group was converted from off-pump to on-pump. The overall 30-day mortality was 1.0%. The mean follow-up was 3.0 +/- 1.3 years. Five-year freedom from death from all causes was 57.7% in the low-EF group and 83.6% in the normal-EF group. The rates of freedom from cardiac death were 73.2% and 93.7%, respectively; and the rates of freedom from the combined endpoint of cardiac death, myocardial infarction, repeat coronary intervention, and heart failure requiring treatment were 65.7% and 77.3%, respectively. CONCLUSION: OPCAB surgery in patients with poor LV function can be performed safely with the same quality in terms of the number of grafts and rate of complete revascularization as in patients with normal LV function. There are higher mortality and cardiac event rates in patients with poor LV function than those with normal LV function at the midterm evaluation after OPCAB.     

Concomitant aortic root remodeling and coronary bypass in a patient with poor left ventricular function.

A 58-year-old man who had previously undergone rectal cancer surgery and who had poor left ventricular function underwent concomitant aortic root remodeling and coronary bypass for aortic root aneurysm with aortic regurgitation and severe coronary artery disease. Intermittent retrograde cold blood cardioplegia and leukocyte-depleted terminal blood cardioplegia were used for myocardial protection. Angiographic studies 1 month after surgery showed improved left ventricular function at an ejection fraction from 24 to 46%. During a 1-year follow-up, he has remained free of any cardiac event. Even though this report is limited to a case and follow-up, this technique is expected to be beneficial even in patients with severely depressed left ventricular function when the postoperative quality of life is considered.