We found a case of hypoplasia of vertebral artery with fibromuscular dysplasia in an 82-yr-old Korean female cadaver during a routine dissection course. In the present case, intracranial hypoplasia in left vertebral artery and bilateral origin of posterior inferior cerebellar artery at the vertebrobasilar junction were recognized. Histopathologically, left vertebral artery showed intimal type of fibromuscular dysplasia both in its extracranial and intracranial courses. These results indicate that the association of fibromuscular dysplasia and hypoplasia does exist in the vertebral artery, although the etiologies are not verified yet.
Aged, 80 and over ; Female ; Fibromuscular Dysplasia/*pathology ; Heart Ventricles/abnormalities/*pathology ; Humans ; Republic of Korea ; Vertebral Artery/*pathology

A 33-year-old man was admitted to our hospital with chest pain and exertional dyspnea. Two-dimensional echocardiography showed prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Thoracoabdominal CT and cardiac magnetic resonance imaging (CMR) revealed situs ambiguous with polysplenia and noncompaction of the left ventricular myocardium. CMR also demonstrated delayed enhancement of the trabeculations located at the apical portion of the left ventricle. The coronary angiogram was normal. This is the first case of noncompaction of the ventricular myocardium associated with situs ambiguous with polysplenia.
Abnormalities, Multiple/*pathology ; Adult ; Echocardiography ; Heart Ventricles/abnormalities ; Humans ; Magnetic Resonance Imaging ; Male ; Myocardium/*pathology ; Spleen/*abnormalities ; Syndrome ; Tomography, X-Ray Computed

OBJECTIVETo explore the perioperative features of surgical treatment in valvular patients with small left ventricle, and investigate the postoperative early structural changes of left ventricle and its correlation with cardiac function.

METHODSA total of 51 patients with small left ventricle underwent mitral valve replacement from January 2003 to August 2008. There were 7 males and 44 females with mean age of (48 +/- 5) years old. The mean pathologic course was (18 +/- 9) years old. The concomitant procedures included aortic valve replacement in 6 cases, coronary artery bypass grafting in 1 case, tricuspid valvular plasty in 48 cases, left atrial thrombi scavenging in 36 cases, and left atrium folding in 42 cases.

RESULTSThe perioperative mortality was 3.9% (2/51). Fourteen patients (27.5%) suffered from severe low-output syndrome in the earlier period postoperatively. Among them, 5 patients needed secondary cardiopulmonary bypass (CPB) to assist circulation, with the result of 1 patient died of weaning off CPB unsuccessfully, 1 patient revived with intra-aortic balloon pump assisted for another 2 d after termination of CPB and another 1 patient died of multiple organ failure (MOF) 10 d later. Eleven cases complicated with MOF. Five patients complicated with ventricular arrhythmia. The echocardiographic examinations showed that the left ventricular dimensions didn't expand significantly postoperatively at 7 to 14 d postoperatively. Left ventricular end-diastolic diameter (LVEDD) was (36.5 +/- 3.2) mm preoperatively and (38.6 +/- 5.3) mm postoperatively (P > 0.05). Preoperative LVEDD index (LVEDDI) was (45.9 +/- 3.8) ml/m(2) and postoperative LVEDDI was (48.2 +/- 7.4) ml/m(2) (P > 0.05). The contract function of left ventricle was improved postoperatively but with no statistical significance. Ejection fraction was 48.6% +/- 6.7% preoperatively and 52.8% +/- 8.3% postoperatively. Left ventricular fraction shortness was 25.5% +/- 3.3% preoperatively and 27.1% +/- 1.3% postoperatively.

CONCLUSIONSFor the patients with small left ventricle usually, the postoperative emphases should be put on the management of low output syndrome. The decreased dimension of left ventricle doesn't expand in the early period after valvular operation.

Adult ; Aged ; Female ; Heart ; physiopathology ; Heart Valve Prosthesis Implantation ; Heart Ventricles ; abnormalities ; Humans ; Male ; Middle Aged ; Myocardium ; pathology ; Postoperative Period ; Retrospective Studies

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.
Arrhythmias, Cardiac ; physiopathology ; prevention & control ; Cardiac Valve Annuloplasty ; methods ; mortality ; Fontan Procedure ; methods ; mortality ; Heart Defects, Congenital ; mortality ; pathology ; surgery ; Heart Ventricles ; abnormalities ; pathology ; surgery ; Humans ; Pulmonary Atresia ; mortality ; pathology ; surgery ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

BACKGROUNDUmbilical cord around neck, a common obstetric complication, affects fetal hemodynamics. Does it influence fetal cardiac functions? The purpose of this study was to investigate the left and right ventricular systolic and diastolic functions of fetuses with umbilical cord around neck in the third trimester by applying velocity vector imaging (VVI).

METHODSThirty-five cases of fetuses with umbilical cord around neck whose gestational ages from 35 to 40 weeks were selected, including 20 cases of umbilical artery ratio of the highest systolic velocity (S) to the lowest diastolic velocity (D) (S/D) < 3.0 and 15 cases of umbilical artery S/D ≥ 3.0, while 20 cases of normal fetuses of 35 - 40 gestational weeks were selected as the control group. The changes in longitudinal velocity, strain, and strain rate of fetal left and right ventricle in systole and diastole in two groups, and the changes in fetal cardiac function under the situation of umbilical cord around neck were analyzed.

RESULTSLongitudinal strain and strain rate overall of fetal left and right ventricle in systole and diastole were less in fetuses with umbilical artery S/D (3)3.0 and umbilical cord around neck than those in fetuses with umbilical artery S/D < 3.0 and those in control group (P < 0.05); there was no significant difference (P > 0.05) in longitudinal strain and strain rate overall of fetal left and right ventricle in systole and diastole between fetuses with umbilical artery S/D < 3.0 and those in control group.

CONCLUSIONSLeft and right ventricular systolic and diastolic dysfunction was detected in fetuses with umbilical cord around neck and umbilical artery S/D (3)3.0. VVI could sensitively respond to cardiac function changes in fetuses with umbilical cord around neck, which provides another valuable method in the evaluation of fetal cardiac function.

Adult ; Female ; Fetus ; abnormalities ; physiopathology ; Gestational Age ; Heart Ventricles ; diagnostic imaging ; pathology ; physiopathology ; Humans ; Myocardium ; pathology ; Pregnancy ; Pregnancy Complications ; Ultrasonography, Prenatal ; Umbilical Arteries ; diagnostic imaging ; pathology ; physiopathology ; Umbilical Cord ; diagnostic imaging ; physiopathology ; Young Adult

OBJECTIVETo analyze the clinical features and outcome of patients with noncompaction of ventricular myocardium (NVM).

METHODSClinical manifestations, electrocardiograms and echocardiographies data were analyzed in 18 patients with NVM. Mean follow-up period was (11 +/- 5) months.

RESULTSThe patients aged from 1.5 to 71 years, 66.7% patients were males, familial history was observed in 2 cases, congestive heart failure was present in 14 cases, thromboembolic event occurred in 1 patient, arrhythmia induced syncopes were diagnosed in 2 patients and 1 patient was asymptomatic. Abnormal electrocardiograms were observed in all patients, including premature ventricular beats (7 cases), heart block (4 cases), and atrial fibrillations (4 cases). Echocardiographies showed that noncompaction of ventricular myocardium localized in the left ventricle in 17 patients, and right ventricle in 1 patient. The extension of noncompaction myocardium was predominantly at the apex (72%). N/C was 2.3 - 3.1. EF was less than 50% in 15 patients. Hypokinetic movements were observed in both noncompacted and compacted segments. During the follow-up, 1 patient with congestive heart failure received heart transplantation. ICD was implanted in one patient due to ventricular tachycardia. One patient suffered from sudden cardiac death.

CONCLUSIONSThe most common clinical presentations of NVM are congestive heart failure, cardiac arrhythmias, and thromboembolism. Echocardiography is considered as the best tool for the diagnosis of NVM. ICD, heart transplantation and anticoagulation therapy could improve the prognosis of patients with NVM in selected cases.

Adolescent ; Adult ; Aged ; Arrhythmias, Cardiac ; diagnosis ; Cardiomyopathies ; diagnosis ; diagnostic imaging ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Failure ; diagnosis ; Heart Ventricles ; abnormalities ; Humans ; Infant ; Male ; Middle Aged ; Myocardium ; pathology ; Young Adult