李建蓉
中国医学科学院阜外医院 超声科
Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is easily misdiagnosed as tetralogy of Fallot (TOF). We herein discuss the echocardiographic features of APVS, compare its two subtypes, and clarify some differences between APVS and TOF. From July 1998 to October 2011, 31 patients diagnosed with APVS at Fuwai Hospital underwent echocardiography, computed tomography, or cardiac angiography. APVS was clinically categorized as either infant-type or child-type. We compared the echocardiographic similarities and differences between APVS and TOF and between the two subtypes of APVS. Although enlargement or aneurysmal dilatation was present in the main pulmonary artery (PA) and its branch in most patients, pulmonary dysplasia or even an absent left PA was found in a few patients. Four important echocardiographic features of APVS useful for distinguishing this syndrome from TOF were (1) absence of the pulmonary valve or presence of pulmonary valve dysplasia, (2) concurrent stenosis and regurgitation at the pulmonary annulus, (3) significant aneurysmal dilatation in the areas of the PAs, and (4) increased rather than decreased PA pressure. 10 patients had infant-type APVS and 21 had child-type APVS. Compared with child-type APVS, infant-type APVS was usually characterized by a lower oxygen saturation, more dilated main PA and right PA, lower aorta-PA ratio, higher diastolic PA pressure, and lower incidence of an absent left PA. Echocardiography is important for diagnosing APVS and distinguishing it from TOF. There are minimal differences in the echocardiographic features between infant-type and child-type APVS.
The international journal of cardiovascular imaging 2015
Left ventricular apical hypoplasia (LVAH) is a rare and newly characterized cardiomyopathy. No systemic and detailed case series were reported earlier. We report five patients diagnosed with left ventricular apical hypoplasia at our institute from 2004 to 2011. All the cases underwent clinical examination, eletrocardiography, chest X-ray, transthoracic echocardiography (TTE), and cardiac magnetic resonance imaging (CMRI). We described the clinical presentations, summarized the imaging characteristics of LV apical hypoplasia, and described the associated cardiac malformations. LVAH is characterized by a truncated, spherical left ventricle, without a normal apex; the true apex is occupied by the right ventricle. In addition, the atrioventricular valve anomalies, LV papillary muscle displacement, interventricular and/or interatrial septal bulge aneurysms and patent ductus arteriosus structures coexisted in these patients. LV apical hypoplasia has distinct appearances that can be easily identified on TTE and CMRI. TTE also could accurately define the associated cardiac abnormalities.
Acta cardiologica 2013
Right pulmonary artery to left atrial fistula (RPA-LAF) is a rare cardiovascular anomaly. There were no systematic and detail represent by echocardiography. We chose the patients who diagnosed with RPA-LAF at Fuwai Hospital from 2000 to 2010. All patients underwent clinical examination, chest roentgenogram, laboratory testing, electrocardiography, transthoracic echocardiography (TTE), contrast echocardiography, and cardiac catheterization. In this article, we summarize the characteristics of the TTE for diagnosing the rare cardiovascular anomaly of RPA-LAF. We undertook a detailed review of their TTE and contrast echocardiography findings to determine the characteristic findings of this condition.
Echocardiography (Mount Kisco, N.Y.) 2013
OBJECTIVE:To analyze the clinical and cardiac imaging characteristics of patients with left ventricular apical hypoplasia (LVAH).METHODS:From January 2008 to January 2012, seven patients [3 male/4 female, age: 6 - 44 (19.9 ± 14.2) years] with LVAH were included in this cohort. Transthoracic echocardiogram was performed in all patients, cardiovascular MRI was performed in 3 patients and cardiovascular CT in another 2 patients. In addition, one LVAH patient underwent cardiac catheterization and angiography examination.RESULTS:Four out of 7 patients complained chest discomfort. Precordial murmur was heard in 3 patients. Atrial fibrillation was evidenced by electrocardiogram in 3 patients. Left ventricular end-diastolic diameter [(57.9 ± 11.6) mm] increased while left ventricule (LV) longitudinal diameter reduced in all patients. Left ventricular systolic function was reduced in 2 patients and mean LVEF was (47.6 ± 17.2)%. The interventricular septum bulged towards the right, and the ventricular septum thickness was (7.3 ± 1.2) mm. The papillary muscles were dominant on the flattened LV anteroapical region. The right ventricle elongated and wrapped around the hypoplastic left ventricular apex, and the dimension of right ventricle was (19.7 ± 7.6) mm. Focal fat replacement of the left ventricular apical wall was evidenced in 5 patients underwent cardiovascular MRI or CT examinations.CONCLUSIONS:Clinical symptoms are non-specific in LVAH patients. Truncated and spherical LV, abnormal origin of papillary muscles in the flattened LV anterior apex and an elongated right ventricle wrapping around the LV apex as well as focal fat replacement of the left ventricular apical wall are typical imaging characteristics of LVAH.
Zhonghua xin xue guan bing za zhi 2012
