Robotically assisted mitral valve repair has proven its efficacy during the last decade. The most suitable approach for patients with difficult anatomies, such as morbid obesity, sternal deformities, cardiac rotation, or vascular anomalies, represents a current challenge in cardiac surgery. Herein, we present the case of a 71-year-old patient affected by severe degenerative mitral valve regurgitation with pectus excavatum and a right aortic arch with an anomalous course of the left subclavian artery who was successfully treated using a Da Vinci–assisted approach.
Aged ; Aorta, Thoracic ; Congenital Abnormalities ; Funnel Chest ; Humans ; Mitral Valve Insufficiency ; Mitral Valve ; Obesity, Morbid ; Subclavian Artery ; Thoracic Surgery

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
Adult ; Aortic Coarctation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Constriction, Pathologic ; Diagnosis ; Female ; Follow-Up Studies ; Heart ; Heart Defects, Congenital ; Humans ; Mitral Valve

OBJECTIVETo investigate the autopsy characteristics, pathologic type, malfomation and genetic characteristics of complete atrioventricular septal defect (CAVSD).

METHODSThirty five cases of CAVSD were collected from Maternal and Child Hospital of Haidian District during Jan.2003 to Jan.2015. Autoptic material, clinical history and chromosome examination were reviewed.

RESULTSAmong 35 cases of CAVSD between 18-38 gestational weeks, there were 26 cases with CAVSD A (74.3%, 26/35), 1 case with CAVSD B (2.8%, 1/35) and 8 cases with CAVSD C (22.8%, 8/35). Only CAVSD malformation was seen in 4 cases (11.4%, 4/35). Multiple malformations were seen in 31 cases (88.6%, 31/35). Combined malformations most frequently occurred in cardiovascular, respiratory and locomotor system. Among 15 cases with chromosome examination, chromosome aberrations was found in 13 cases (13/15) and trisomy-21 was found in 11 cases (11/15).

CONCLUSIONSCAVSD is a rare disease and CAVSD A is the most common type. CAVSD is usually combined with other malformations and chromosome aberrations.

Abnormalities, Multiple ; genetics ; pathology ; Autopsy ; Chromosome Aberrations ; Gestational Age ; Heart Septal Defects ; Humans ; Mitral Valve Insufficiency ; genetics ; pathology

Ellis-van Creveld (EvC) syndrome is a rare autosomal recessive malformation disorder. Cardiac defects are observed in about 50% of EvC cases. Surgical data is lacking on the prognosis and life expectancy of EvC patients. Herein, we report the case of a 38-year-old man with EvC syndrome who underwent two surgical corrections for cardiac anomalies. This report supplements the available information on the clinical course of EvC syndrome in older patients.
Adult ; Ellis-Van Creveld Syndrome ; diagnosis ; genetics ; surgery ; Genes, Recessive ; Hand Deformities ; complications ; Humans ; Life Expectancy ; Male ; Mitral Valve Insufficiency ; surgery ; Prognosis ; Quality of Life ; Tooth Abnormalities ; complications

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

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.
Abnormalities, Multiple ; Aortic Coarctation ; Aortic Valve Stenosis ; Cause of Death ; Counseling ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Genetic Testing ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Hypoplastic Left Heart Syndrome ; Intellectual Disability ; Mitral Valve Stenosis ; Prevalence ; Retrospective Studies

OBJECTIVETo investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).

METHODData of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up.

RESULTMost patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure.

CONCLUSIONThe accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.

Bland White Garland Syndrome ; Cardiac Output, Low ; Cardiopulmonary Bypass ; Coronary Vessel Anomalies ; surgery ; Echocardiography ; Electrocardiography ; Extracorporeal Membrane Oxygenation ; Humans ; Infant ; Mitral Valve Insufficiency ; Perioperative Care ; methods ; Pulmonary Artery ; abnormalities ; Retrospective Studies ; Ventricular Function, Left

OBJECTIVETo investigate the clinical manifestations and treatment of congenital atresia of the left main coronary artery (CLMCA-A).

METHODFour patients were diagnosed to have CLMCA-A from June 2010 to June 2012 in Beijing Anzhen Hospital. Clinical manifestations, ultrasound, ECG and angiographic characteristics were analyzed and summarized.

RESULTOf the 4 cases, age of onset was 3 months to 2 yrs. Three cases were diagnosed by angiography, and 1 case by CTA . All 4 cases had chronic heart failure symptoms and signs, such as sweating, shortness of breath, easily choked by milk, predispose to pneumonia, activity intolerance. ECG showed abnormal Q wave and other ischemic signs such as ST-T segment depression. Ultrasonography showed left ventricular enlargement, left ventricular systolic function was normal or slightly reduced, and there was moderate to large amount of mitral valve regurgitation. Left ventricular trabeculations increased. Coronary collateral circulation increased. Left coronary artery appeared to be slender and disconnected with left coronary artery sinus. Aortic root angiography was the golden diagnostic standard. Angiography was performed in 3 patients and showed that left main coronary artery had a blind end, diameter 1.1-2.0 mm. The right coronary artery was found rising from the right coronary sinus and visible on coronary collateral circulation. Contrast agent developing sequence: right coronary artery-collateral vessels-left coronary artery distal branches-left main coronary artery. CTA exam was performed in 2 cases and in 1 case the diagnoses was confirmed. All the 4 patients are currently in the close follow-up, digoxin and diuretics were taken everyday and clinical symptoms were improved.

CONCLUSIONCLMCA-A is not rare, its clinical manifestations should be differentiated from those of cardiomyopathy, endocardial fibroelastosis, congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc. For pediatric patients with cardiac enlargement, abnormal heart function, mitral valve regurgitation etc, attention must be paid to consider the developmental abnormality of coronary artery, particularly the CLMCA-A diagnosis.

Child ; Child, Preschool ; Coronary Angiography ; methods ; Coronary Vessel Anomalies ; diagnosis ; pathology ; Coronary Vessels ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Endocardial Fibroelastosis ; diagnosis ; pathology ; Female ; Heart Defects, Congenital ; diagnosis ; pathology ; Humans ; Infant ; Male ; Mitral Valve Insufficiency ; diagnosis ; pathology ; Pulmonary Artery ; abnormalities ; diagnostic imaging ; Tomography, X-Ray Computed ; methods

OBJECTIVETo summarize the clinical characteristics, surgical management and postoperative complications in patients with congenital bicuspid aortic valve (CBAV) over 50 years of age.

METHODSFrom January 2009 to September 2011, 73 CBAV patients aged 51-76 years (mean 61.8∓0.73 years) were treated in our center. Except for 1 patient who underwent Bentall surgery and another having Wheat surgery, all the patients received aortic valve replacement (AVR), including 7 with double (mitral and aortic) valve replacement (DVR), 6 with mitral valvular plasty, 11 with tricuspid valvular plasty, 8 with coronary artery bypass graft implantation, 1 with aortic-left ventricular tunnel repair, 1 with atrial maze ablation, and 1 with left atrial thrombosis removal.

RESULTSTwo patients died after the surgery, with a perioperative mortality rate of 2.7%. The cardiopulmonary bypass time was 78-217 min (mean 131.9 ∓6.0 min) with an aortic blocking time of 56-158 min (mean 88.2 ∓4.8 min) and total postoperative ICU time of 23.0-647.4 h (mean 97.9∓10.5 h). The postoperative complications included low heart output syndrome in 5 cases, bleeding in 4 cases, wound debridement in 4 cases, and hemodialysis due to acute renal failure in 1 case. The left ventricular end diastolic diameter reduced significantly after the surgery (52.6∓1.7 vs 43.2∓1.0, P=0.001). No significant changes were detected in the left ventricular ejection fraction (62.3∓2.5 vs 65.5∓1.3, P=0.257).

CONCLUSIONThorough preoperative examination, preoperative risk factor assessment, timely perioperative interventions, careful evaluation of patients' tolerance of surgery, and prevention of surgical complications are essential to decrease the perioperative mortality in elderly patients with CBAV.

Aged ; Aortic Valve ; abnormalities ; surgery ; Coronary Artery Bypass ; Female ; Heart Valve Diseases ; mortality ; surgery ; Heart Valve Prosthesis Implantation ; methods ; Humans ; Male ; Middle Aged ; Mitral Valve ; surgery ; Postoperative Complications ; prevention & control ; Retrospective Studies

OBJECTIVETo summarize the echocardiographic features of a wide spectrum of congenital mitral valve anomalies.

METHODSThe medical records, echocardiograms, cardiac catheterization studies, and surgical reports were reviewed. The mitral valve anomalies evaluated in the study included parachute mitral valve, double orifice mitral valve, congenital mitral stenosis with 2 papillary muscles, anomalous papillary muscle rotation, and 3 commissures and papillary muscles in 15 cases. Surgeries were performed in 11 patients, and 1 patient underwent transcatheter closure of the patent ductus arteriosus.

RESULTSThe echocardiograms of 6 cases of parachute mitral valve were characterized by a hypoplastic mitral valve with short chordal attachments to a single posterior medial papillary muscle. The mitral valve demonstrated restricted motion. The double orifice mitral valve were featured by two separate mitral valve orifice, with each suborifice supported by its own tension apparatus in 4 patients. Asymmetric hypoplastic mitral valve stenosis with two papillary muscles was found in 1 patient with short and unbalanced chordal attachments to the anterior lateral major papillary muscle. Anomalous papillary muscle rotation was found in 1 patient. Mirtal anomaly with 3 major commissures and 3 papillary muscles was found in 3 patients.

CONCLUSIONSEchocardiography offers clear demonstration of the mitral valve thickness and chordal attachments, and allows visualization of the position and the number of the papillary muscles and interpapillary spaces for evaluation of the mobility of the valve leaflets, therefore can be a valuable diagnostic modality for congenital mitral valve anomalies, especially congenital mitral stenosis.

Adolescent ; Adult ; Child ; Child, Preschool ; Echocardiography, Doppler, Color ; Female ; Humans ; Infant ; Middle Aged ; Mitral Valve ; abnormalities ; diagnostic imaging ; Mitral Valve Stenosis ; congenital ; diagnostic imaging ; Papillary Muscles ; abnormalities ; diagnostic imaging ; Young Adult