Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.
Adult ; Aortic Valve Stenosis/*complications/diagnosis ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Humans ; Hypoplastic Left Heart Syndrome/*complications/diagnosis ; Magnetic Resonance Imaging, Cine ; Male ; Pulmonary Valve Stenosis/*complications/diagnosis

Aortic Valve Stenosis ; complications ; diagnosis ; Female ; Humans ; Middle Aged ; Syncope ; diagnosis ; etiology

INTRODUCTIONThe treatment of aortic valve stenosis (AS) is seeing renewed interest mainly due to the availability of transcatheter therapies. However, the number of epidemiological studies of this disease in Singapore is limited. We aimed to describe the aetiology and clinical presentation of AS in Singapore, as well as patients' attitudes toward it. Our findings may facilitate the future planning and utilisation of resources to better manage these patients.

METHODS249 consecutive patients who underwent transthoracic echocardiography (from April 1999 to April 2008) and diagnosed with severe AS were assessed. Demographic and clinical data were collected, and patients' decisions on surgery were determined.

RESULTSThe mean patient age was 71 (range 23-98) years. 50.2% of patients were male. The commonest presenting symptom was dyspnoea, and 40 (16.0%) patients had coexistent atrial fibrillation. The aetiology of AS was degenerative in 216 (86.7%), rheumatic in 11 (4.4%) and related to a bicuspid valve in 22 (8.9%) patients. The average peak velocity across the aortic valve was 4.2 ± 0.8 m/s and the mean aortic valve area was 0.76 ± 0.13 cm2. The overall mean logistic EuroSCORE was 10.7 ± 12.3. 105 (42.2%) patients who were offered surgery refused. 87 (35%) deaths were seen during the follow-up period (mean duration 14.5 months), which also saw 68 (27%) patients undergo surgery and 86 (34%) patients hospitalised for heart failure.

CONCLUSIONDegenerative AS was the commonest aetiology in this contemporary cohort of patients. Despite the known benefits of surgery, the refusal rate for surgery remained high.

Adult ; Aged ; Aged, 80 and over ; Aortic Valve ; pathology ; Aortic Valve Stenosis ; complications ; diagnosis ; epidemiology ; Asian Continental Ancestry Group ; Atrial Fibrillation ; complications ; diagnosis ; Cohort Studies ; Dyspnea ; complications ; diagnosis ; Echocardiography ; methods ; Electrocardiography ; methods ; Female ; Humans ; Male ; Middle Aged ; Singapore

A 68-year-old woman with known severe aortic stenosis was admitted to the hospital because of hematochezia and dizziness. She had received several blood transfusions over the preceding 3 years and undergone right hemicolectomy 2 years ago for severe lower gastrointestinal bleeding. Postoperative histology revealed angiodysplasia involving the ascending colon. After the hemicolectomy, she continued to have hematochezia and anemia and required additional blood transfusions for anemia. During this admission, platelet count, activated partial-thromboplastin time, von Willebrand factor antigen, and von Willebrand factor ristocetin cofactor were normal. She had a severe deficiency of high-molecular-weight multimers of von Willebrand factor. Colonoscopy showed angiodysplasia in the transverse colon at this time. Successful coagulation of the bleeding angiodysplasia was achieved by argon plasma coagulator. No additional bleeding was observed thereafter. We report a case of Heyde's syndrome with abnormal von Willebrand factor in a patient who presented with intestinal angiodysplasia and aortic stenosis.
Aged ; Anemia/etiology/therapy ; Angiodysplasia/*complications ; Aortic Valve Stenosis/*complications ; Colonic Diseases/*complications ; English Abstract ; Female ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Syndrome ; von Willebrand Disease/*complications/diagnosis

Implanting a valve that will reduce left ventricular mass is critical in aortic stenosis. Regression of left ventricular hypertrophy in 46 aortic valve replacement (AVR) patients receiving a St. Jude Medical (SJM) valve was assessed by serial electrocardiographic and echocardiographic studies during the preoperative, immediate, and late postoperative periods. The patients were divided into three groups according to valve size; 19 mm group (n=9), 21 mm group (n=20), and 23+mm group (n=17). There was no surgical mortality. The NYHA functional class improved from an average of 2.2+/-0.8 preoperatively to 1.3+/-0.5 post-operatively. Left ventricular muscle mass index (LVMI) regression failed to reach statistical significance in the 19 mm group, whereas in the other two groups a steady decrease in the LVMI occurred with follow up. ECG findings were less remarkable showing insignificant differences in voltage among the three groups (p=0.000). In conclusion, the current data suggest that the 19 mm SJM valve may not result in satisfactory left ventricular muscle mass regression despite adequate function, even in small patients. Therefore, additional procedures to accommodate a larger valve may be warranted in the aortic annulus smaller than 21 mm.
Adult ; Aged ; Aortic Valve/ultrasonography ; Aortic Valve Stenosis/surgery* ; Aortic Valve Stenosis/complications ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Valve Prosthesis* ; Human ; Hypertrophy, Left Ventricular/prevention & control* ; Hypertrophy, Left Ventricular/etiology ; Hypertrophy, Left Ventricular/diagnosis ; Male ; Middle Age ; Multivariate Analysis ; Postoperative Period ; Remission Induction ; Risk Factors ; Treatment Outcome

Aortic Valve Stenosis ; blood ; diagnosis ; etiology ; Child ; Coronary Artery Disease ; blood ; diagnosis ; etiology ; Coronary Vessels ; diagnostic imaging ; pathology ; Echocardiography ; Female ; Humans ; Hyperlipoproteinemia Type II ; blood ; complications ; diagnosis ; genetics ; Lipoproteins, LDL ; blood ; Triglycerides ; blood

Little is known regarding long-term survival and changes in systolic function following surgery after the occurrence of a severe left ventricular (LV) dysfunction in patients with severe aortic stenosis. Inclusion criteria were an aortic valve area less than 1 cm2 and an LV ejection fraction (EF) less than 35%. Between January 1990 and July 2007, 41 (male: 30) patients were identified. The pre-operative mean EF and mean aortic valve area were 26.7+/-6.1% and 0.54+/-0.2 cm2, respectively. Concomitant coronary artery bypass surgery was performed in 8 patients (19.6%). Immediate post-operative echocardiogram showed to be much improved in LV EF (27.2+/-5.5 vs. 37.4+/-11.3, P<0.001), LV mass index (244.2+/-75.3 vs. 217.5+/-71.6, P=0.006), and diastolic LV internal diameter (62.5+/-9.3 vs. 55.8+/-9.6, P<0.001). Post-operative LV changes were mostly complete by 6 months, and were maintained thereafter. There was one in-hospital mortality (2.4%) and 12 late deaths including one patient diagnosed with malignancy in whom LV function was normal. Multivariate analysis showed pre-operative atrial fibrillation and NYHA FC IV to be significant risk factors for cardiac-related death. Aortic valve replacement in patients with significant aortic stenosis and severe LV dysfunction showed acceptable surgical outcomes. Moreover, LV function improved significantly in many patients.
Adult ; Aged ; Aortic Valve/*surgery ; Aortic Valve Stenosis/complications/*mortality/surgery ; Atrial Fibrillation/diagnosis ; Coronary Artery Bypass/methods ; Coronary Artery Disease/diagnosis ; Echocardiography ; Hospital Mortality ; Humans ; Male ; Middle Aged ; Risk Factors ; Severity of Illness Index ; Treatment Outcome ; Ventricular Dysfunction, Left/complications/*mortality/surgery