Marfan syndrome is infrequently diagnosed early in infancy. The morphologic characteristics and prognosis in infantile Marfan syndrome may be quite different from those in older patients. Characteristic cardiac findings in early life include mitral valve prolapse, valvular regurgitation, and aortic root dilation. Morbidity and mortality may be high for infants diagnosed with Marfan syndrome. Cardiac surgery for cardiovascular complication in infants often resulted in mortality due to the patient's young age and small size of valve. But recently, some reports say that operations for cardiovascular complications of Marfan syndrome can be performed in children with low operative morbidity and mortality. Echocardiographic findings of this patient were mitral valve prolapse, severe mitral regurgitation and aortic root dilatation. She underwent mitral valve replacement due to severe mitral valve prolapse and mitral regurgitation, and will have an aortic root and valve replacement in the near future. We experienced a case of infantile Marfan syndrome diagnosed by echocardiographic findings, and reported the case with associated literature.
Child ; Dilatation ; Echocardiography ; Humans ; Infant ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Mortality ; Prognosis ; Thoracic Surgery

BACKGROUND: Minimally invasive cardiac surgery appears to offer certain advantages such as earlier postoperative recovery and a greater cosmetic effect than that achieved through conventional sternotomy. However, this approach has not yet been widely adopted in Korea to replace complex open heart surgery such as mitral valve reconstruction. This study compared the results of robot assisted minimally invasive mitral valve repair with those results of conventional sternotomy. MATERIAL AND METHOD: From December 1993 to December 2005, 520 consecutive patients underwent mitral valve reconstruction for mitral regurgitation in our institution. These patients were subdivided according to those whose surgery used the conventional sternotomy approach (Group S, n=432) and those who underwent minimally invasive right anterior thoracotomy (Group M, n=88); we then compared the clinical results of both groups. When we performed minimally invasive right thoracotomy, we used a robot (AESOP 3000) and made an incision less than 5 cm. RESULT: Our study patients in both groups were similar for their age, gender and preoperative ejection fraction. There were two hospital mortalities in group S. but there was no mortality in the group M patients. Significant reductions in the ICU stay and the postoperative hospital stay were observed in the group M patients compared with the group S patients. However, both the bypass time and the aortic cross-clamp time were significantly longer in the group M patients. In spite of the confined incision in the group M patients, there were no limitations on the mitral valve repair techniques. There was a similar frequency of postoperative significant residual mitral regurgitation in both groups. CONCLUSION: In this study, the minimally invasive mitral valve repair showed comparable early results with the conventional sternotomy patients. We will now need long-term follow-up of these patients who underwent minimally invasive mitral valve repair, but we anticipate that based on the results of this study, we will begin to routinely perform minimally invasive cardiac surgery as our primary approach for mitral valve reconstruction.
Follow-Up Studies ; Hospital Mortality ; Humans ; Korea ; Length of Stay ; Mitral Valve Insufficiency ; Mitral Valve* ; Mortality ; Sternotomy* ; Surgical Procedures, Minimally Invasive ; Thoracic Surgery ; Thoracotomy

Heart Aneurysm ; mortality ; pathology ; surgery ; Humans ; Mitral Valve Insufficiency ; mortality ; physiopathology ; surgery ; Myocardial Infarction ; mortality ; physiopathology ; surgery ; Ventricular Dysfunction, Left ; pathology ; surgery

Management of ischemic mitral regurgitation (MR) is challenging. The aim of this study was to investigate long-term clinical and echocardiographic results of restrictive mitral annuloplasty for ischemic MR. From 2001 through 2010, 96 patients who underwent myocardial revascularization with restrictive mitral annuloplasty using a vascular strip for ischemic MR were analyzed. Patients were stratified into two groups based on left ventricular ejection fraction (LVEF): group I, n = 50, with LVEF > 35% and group II, n = 46, with LVEF < or = 35%. The early mortality rate was 2.1% (2/96) and the late cardiac mortality rate was 11.5% (11/96). MR grade was reduced at discharge (0.8 +/- 0.7) but increased during follow-up (1.1 +/- 0.8, P = 0.001). There was no intergroup difference in terms of freedom from recurrent MR > or = moderate eight years after surgery (94.1% +/- 5.7%, group I vs 87.8% +/- 7.2%, group II; P = 0.575). NYHA functional class (odds ratio [OR], 2.2; P = 0.044) and early postoperative residual MR > or = mild (OR, 25.4; P < 0.001) were independent predictors of recurrent MR. Restrictive mitral annuloplasty using a vascular strip is effective in ischemic MR. It is important to avoid early postoperative residual MR.
Aged ; Aged, 80 and over ; Coronary Artery Disease/mortality/*surgery ; Echocardiography ; Female ; Follow-Up Studies ; Heart Valve Prosthesis Implantation ; Humans ; Male ; Middle Aged ; Mitral Valve/physiopathology/*surgery ; Mitral Valve Annuloplasty/*methods ; Mitral Valve Insufficiency/mortality/*surgery ; Myocardial Ischemia/mortality/*surgery ; Myocardial Revascularization ; Stroke Volume ; Treatment Outcome ; Vascular Surgical Procedures

We compared clinical (30+/-24 months) and echocardiographic follow-up (22 +/- 20 months) data of 184 consecutive patients with myxomatous degenerative mitral regurgitation (Group A) and 85 consecutive patients with rheumatic mitral regurgitation (Group B) after repair. Selection criteria for rheumatic etiology was predominant mitral regurgitation with valve area >or= 2.0 cm2 and with no significant calcification in valvular apparatus. Repair was successful in 93% of group A and in 92% of group B (p>0.05). There was no difference of operative mortality (1% vs 0%) and of the incidence of the second-pump valve replacement (4% vs 5%). The 4-yr survival, 4-yr event-free survival, and 4-yr mitral regurgitation-free survival rates in group A were 96 +/- 2%, 89 +/- 4%, and 76 +/- 5%, respectively, which were not different from those in group B (97 +/-2 %, 93 +/- 4%, and 68 +/-7 %, p >0.05). Independent determinants of development of at least moderate regurgitation in group A were no use of ring annuloplasty (hazards ratio 6.6, 95% CI 2.0 to 21.5) and new chordae formation (hazards ratio 3.5, 95% CI 1.4 to 8.7). In group B, no use of ring annuloplasty (hazards ratio 15.3, 95% CI 3.5 to 66.7) also was independent predictor. Valve repair is highly feasible in selected patients with rheumatic mitral regurgitation, and clinical course is not significantly different from that of patients with degenerative mitral regurgitation.
Adult ; Comparative Study ; Disease-Free Survival ; *Echocardiography ; Female ; Follow-Up Studies ; Human ; Male ; Middle Aged ; Mitral Valve Insufficiency/etiology/mortality/*surgery/*ultrasonography ; Rheumatic Heart Disease/mortality/*surgery/*ultrasonography ; Survival Analysis ; Treatment Outcome

Acute abdominal Aortic occlusion is rare but it is a vascular emergency with high mortality and morbidity. Therefore, delay in diagnosis can have severe impact on the prognosis. A 60-year-old women complained of paresthesia, paralysis, and severe pain in bilateral lower extremities on 13th day after open heart surgery for mitral stenosis, atrial fibrillation, coronary arterial stenosis, tricuspid regurgitation, and atrial septal defect. Her skin was mottled and cool from the umbilicus to the feet, and there were no palpable pulses in the lower exteremities. We diagnosed an acute abdominal aortic occlusion using the Multi-Detector Row Spiral Computed Tomography and successfully treated the problem with emergent thrombo-embolectomy and Aortobifemoral bypass.
Aorta, Abdominal ; Atrial Fibrillation ; Constriction, Pathologic ; Diagnosis ; Emergencies ; Female ; Foot ; Heart Septal Defects, Atrial ; Heart* ; Humans ; Lower Extremity ; Middle Aged ; Mitral Valve Stenosis ; Mortality ; Paralysis ; Paresthesia ; Prognosis ; Skin ; Thoracic Surgery* ; Thrombosis ; Tomography, Spiral Computed ; Tricuspid Valve Insufficiency ; Umbilicus

OBJECTIVETo investigate the role of left atrial appendage (LAA) closure for cerebral ischemic stroke prevention following mitral valve replacement.

METHODSRetrospective data on 860 consecutive adult patients undergoing mitral valve replacement between January 2008 and January 2013 were analyzed. There were 414 male and 446 female patients, with a mean age of (53 ± 12) years. The patients were divided into two groups according to whether the left atrial appendage was closed during operation: LAA closure group (n = 521) and non-LAA closure group (n = 339).Early mortality, postoperative cerebral ischemic stroke and the risk factors for cerebral ischemic stroke were assessed. Multivariate analysis was performed using logistic regression analysis.

RESULTSCompared with non-LAA closure group, LAA closure group had higher proportion of female gender, higher percentage of patients with cardiac insufficiency, pulmonary hypertension and left atrial thrombus, higher incidence of mechanical valve implantation and concurrent tricuspid surgery, and larger preoperative diameter of left atrium, but lower proportion of hypertension and patients undergoing coronary artery bypass surgery, and shorter aorta cross clamping time (χ² = 6.807 to 122.576, t = -2.818 and 3.756, all P < 0.05). There were no differences in exploratory thoracotomy for bleeding and in-hospital mortality between the two groups. Postoperative cerebral ischemic stroke occurred in 12 patients (1.4%). The incidence of cerebral ischemic stroke in LAA closure group was significantly lower than in non-LAA closure group (0.6% vs.2.7%, χ² = 6.452, P = 0.011).Logistic regression analysis showed that LAA closure was a significant protective factor for postoperative cerebral ischemic stroke (OR = 0.189, 95% CI: 0.039 to 0.902, P = 0.037) while history of cerebrovascular disease (OR = 4.326, 95% CI:1.074 to 17.418, P = 0.039) and preoperative diameter of left atrium (OR = 1.509, 95% CI: 1.022 to 1.098, P = 0.002) being the independent risk factors for postoperative cerebral ischemic stroke. The subgroup analysis showed that, for atrial fibrillation patients, LAA closure was a strong protective factor (OR = 0.064, 95% CI: 0.006 to 0.705, P = 0.025), but LAA closure was not a significant predictive factor (OR = 1.902, 95% CI: 0.171 to 21.191, P = 0.601) in non-atrial fibrillation patients.

CONCLUSIONConcurrent LAA closure during mitral valve replacement is safe and effective to reduce the early postoperative risk of cerebral ischemic stroke in atrial fibrillation patients.

Adult ; Aged ; Atrial Appendage ; surgery ; Atrial Fibrillation ; Brain Ischemia ; complications ; prevention & control ; Coronary Artery Bypass ; Female ; Heart Valve Prosthesis Implantation ; Hospital Mortality ; Humans ; Incidence ; Male ; Middle Aged ; Mitral Valve ; Mitral Valve Insufficiency ; surgery ; Nervous System Diseases ; Retrospective Studies ; Risk Factors ; Stroke ; prevention & control ; Thrombosis

OBJECTIVETo review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.

METHODSThere were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.

RESULTSPostoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.

CONCLUSIONSAlthough late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.

Aged ; Aorta ; Bland White Garland Syndrome ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Heart ; Heart Failure ; Hospital Mortality ; Humans ; Mitral Valve Insufficiency ; Perioperative Period ; Postoperative Care ; Prostheses and Implants ; Pulmonary Artery ; abnormalities ; Reoperation ; Treatment Outcome ; Ventricular Function, Left

BACKGROUND: This study was undertaken to analyze the outcome of composite valve graft replacement(CVGR) for the treatment of aneurysms of the ascending aorta involving the aortic root. MATERIAL AND METHOD: Between April 1995 and June 2001, 56 patients had replacement of the ascending aorta and aortic root with a composite graft valve and were reviewed retrospectively. Aortic regurgitation was present in 50 patients(89%), Marfan's syndrome in 18 patients(32%), and bicuspid aortic valve in 7(12.5%). The indications for operation were annuloaortic ectasia(AAE) in 30 patients(53.6%), aortic dissection in 13(23.2%), aneurysms of the ascending aorta involving aortic root in 11(19.6%), and aortitis in 2(3.6%). Cardiogenic shock due to the aortic rupture was present in 2 patients. Nine patients(16%) had previous operations on the ascending aorta or open heart surgery. The operative techniques used for CVGR were the aortic button technique in 51 patients(91%), the modified Cabrol technique in 4, and the classic Bentall technique in 1. The concomitant procedures were aortic arch replacement in 24 patients(43%), coronary artery bypass graft in 8(14.3%), mitral valve repair in 2, redo mitral valve replacement in 1, and the others in 7. The mean time of circulatory arrest, total bypass, and aortic crossclamp were 21+/-14 minutes, 186+/-68 minutes, and 132+/-42 minutes, respectively. RESULT: Early mortality was 1.8%(1/56). The postoperative complications were left ventricular dysfunction in 16 patients(28.6%), reoperation for bleeding in 7(12.5%), pericardial effusion in 2, and the others in 7. Fifty-three patients out of 55 hospital survivors were followed up for a mean of 23.2+/-18.7 months(1-75 months). There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage, and CVGR-related late mortality was 1.9%. The 1- and 6-year actuarial survival was 98.1+/-1.9% and 93.2+/-5.1%, respectively. Two patients required reoperation for complication of CVGR(3.8%) and two other patients required subsequent operations for dissection of the remaining thoracoabdominal aorta. The 1- and 6-year actuarial freedom from reoperation was 97.8+/-2.0% and 65.3+/-26.7%, respectively. CONCLUSION: This study suggests that aortic root replacement with a composite valve graft for a variety of aneurysms of the ascending aorta involving the aortic root is a safe and effective therapy, with good early and intermediate results. Careful follow-up of all patients following composite graft root replacement is important to long-term survival.
Aneurysm ; Aorta ; Aorta, Thoracic ; Aortic Rupture ; Aortic Valve ; Aortic Valve Insufficiency ; Aortitis ; Bicuspid ; Cerebral Hemorrhage, Traumatic ; Coronary Artery Bypass ; Follow-Up Studies ; Freedom ; Hemorrhage ; Humans ; Marfan Syndrome ; Mitral Valve ; Mortality ; Pericardial Effusion ; Postoperative Complications ; Reoperation ; Retrospective Studies ; Shock, Cardiogenic ; Survivors ; Thoracic Surgery ; Transplants* ; Ventricular Dysfunction, Left

The prognostic value of the left ventricle ejection fraction (LVEF) after acute myocardial infarction (AMI) has been questioned even though it is an accurate marker of left ventricle (LV) systolic dysfunction. This study aimed to examine the prognostic impact of LVEF in patients with AMI with or without high-grade mitral regurgitation (MR). A total of 15,097 patients with AMI who received echocardiography were registered in the Korean Acute Myocardial Infarction Registry (KAMIR) between January 2005 and July 2011. Patients with low-grade MR (grades 0-2) and high-grade MR (grades 3-4) were divided into the following two sub-groups according to LVEF: LVEF < or = 40% (n = 2,422 and 197, respectively) and LVEF > 40% (n = 12,252 and 226, respectively). The primary endpoints were major adverse cardiac events (MACE), cardiac death, and all-cause death during the first year after registration. Independent predictors of mortality in the multivariate analysis in AMI patients with low-grade MR were age > or = 75 yr, Killip class > or = III, N-terminal pro-B-type natriuretic peptide > 4,000 pg/mL, high-sensitivity C-reactive protein > or = 2.59 mg/L, LVEF < or = 40%, estimated glomerular filtration rate (eGFR), and percutaneous coronary intervention (PCI). However, PCI was an independent predictor in AMI patients with high-grade MR. No differences in primary endpoints between AMI patients with high-grade MR (grades 3-4) and EF < or = 40% or EF > 40% were noted. MR is a predictor of a poor outcome regardless of ejection fraction. LVEF is an inadequate method to evaluate contractile function of the ischemic heart in the face of significant MR.
Aged ; Coronary Angiography ; Coronary Artery Disease/mortality/*pathology/surgery ; Echocardiography ; Female ; Heart/radiography ; Humans ; Male ; Middle Aged ; Mitral Valve Insufficiency/*pathology ; Myocardial Infarction/mortality/*pathology/surgery ; Myocardium/pathology ; Percutaneous Coronary Intervention ; Prospective Studies ; Stroke Volume/*physiology ; Treatment Outcome ; Ventricular Dysfunction, Left/*surgery ; Ventricular Function, Left/physiology