先天性主动脉瓣上狭窄的外科治疗

目的总结6例先天性主动脉辫上狭窄（SVAS）术前诊断和外科治疗中的经验、教训和疗效。方法2003年1月至2008年6月收治SVAS患者6例，男性3例，女性3例，年龄1．9-19．0（5．73±6．36）岁，局限性狭窄5例，弥漫性狭窄1例。Williams综合征3例，3例并发其他心脏畸形，其中1例同时合并动脉导管未闭、主动脉瓣下狭窄、主动脉瓣两叶畸形、二尖瓣狭窄关闭不全和细菌性心内膜炎，1例合并动脉导管未闭，还有1例合并肺动脉狭窄。术前超声心动图检查均有初步诊断，有2例患者手术前做了左心导管和造影，3例行64排螺旋CT扫描，对诊断和手术方案的确定很有意义。手术前检查发现跨狭窄环的压差为45-121mmHg。手术方法：单片法加宽3例，倒“Y”法3例，同期行主动脉瓣置换、二尖瓣置换、左室流出道疏通和动脉导管结扎1例，行肺动脉加宽1例，行动脉导管缝闭1例。结果本组手术无死亡，除合并细菌性心内膜炎及多种畸形的患者术后出现低心排和呼吸功能不全和感染，其余患者恢复顺利，所有患者痊愈出院。手术后有1例虽然主动脉瓣上狭窄解除理想，但是发现主动脉弓有弥漫性轻度狭窄。结论SVAS经术前超声心动图检查基本可以诊断，对Williams综合征患者术前应进行更为详细的检查，如果有条件应该考虑行无创伤的64排螺旋CT检查，可以发现合并的畸形如主动脉弓的病变，肺动脉狭窄等；一旦发现主动脉辫上狭窄应该积极手术治疗，否则可能引起心律失常和猝死。SVAS的手术治疗效果良好，目前不主张应用单片法进行矫治。     

Left ventricular apical aneurysm in cardiac sarcoidosis

A 53-year-old woman was hospitalized for general fatigue and palpitations. An electrocardiogram showed ST elevation and T wave inversion in leads II, III, aVF, and V4-6. Cardiac catheterization was performed since the echocardiogram demonstrated the existence of a left ventricular apical aneurysm. Left ventriculography showed an aneurysm of the apex. An endomyocardial biopsy specimen from the left ventricular apical wall demonstrated typical noncaseating granulomas with giant cells. The patient was diagnosed as having cardiac sarcoidosis. There was no evidence suggesting involvement of other systemic organs. Cardiac sarcoidosis should be considered within a spectrum of diseases that cause left ventricular aneurysm.     

Results of surgery for congenital supravalvular aortic stenosis.

Of eight children aged 3 to 15 years with surgical correction of severe supravalvular aortic stenosis, 6 were evaluated 7 to 44 months later by repeat cardiac catheterization and aortography. Prosthetic patch angioplasty was performed in all cases. Preoperative systolic gradients ranged from 40 to 90 mm Hg (average 70); postoperative gradients ranged from 0 to 20 mm Hg (average 11). The postoperative anglographic appearance of the ascending aorta was near normal in all six patients, and none had new aortic valve insufficiency. These results of surgery for supravalvular aortic stenosis are judged to be excellent.     

Congenital tubular supravalvular aortic stenosis with massive coronary artery dilatation in a 35-year-old man

Supravalvular aortic stenosis is a rare cause of left ventricular outflow obstruction in adults. It occurs as an isolated defect sporadically or on a hereditary basis with an autosomal dominant trait without further phenotypical anomalies, or as part of the Williams syndrome with mental retardation and multiple other anomalies. This lesion was proved to result from a defect of the elastin coding gene. Supravalvular aortic stenosis is frequently associated with cardiovascular defects, particularly of the peripheral pulmonary arteries, thoracic aorta, carotid, subclavian, and coronary arteries and the aortic valve. The coronary arteries are subject to an increased perfusion pressure leading to dilatation, tortuosity and accelerated arteriosclerosis. We give details of a 35-year-old patient in whom a previously asymptomatic supravalvular aortic stenosis is associated with an excessive dilatation of the right coronary artery and the left anterior descending coronary artery as well as an ostium stenosis of the left common carotid artery. The patient did not present any phenotypical anomalies of the Williams syndrome.     

Giant left ventricular aneurysm and aortic stenosis in a newborn

Congenital ventricular aneurysm (CVA) is a rare, poorly understood and potentially lethal cardiac anomaly. We report a neonatal case, remitted to our hospital due to a cardiogenic shock. Echocardiographic study revealed a severe aortic stenosis and a giant left ventricular aneurysm. Surgical intervention was carried out with extracorporeal circulation support, but without cardiac arrest, achieving aortic valvulotomy and aneurismal resection. The postoperative course was uneventful during the first 24 h, but on the second day, the patient presented a sudden unexpected cardiac arrest, not responding to the resuscitation manoeuvres.     

Left ventricular diastolic filling in valvular aortic stenosis

The pattern of left ventricular diastolic filling in patients with valvular aortic stenosis (AS) as assessed by gated blood pool scintigraphy has received little attention. Twenty-one normal persons (group 1), 24 patients with significant AS and ejection fractions of 50% or more (group 2) and 15 patients with significant AS and ejection fractions less than 50% (group 3) were studied. From the time-activity curve, the peak filling rate and mean filling rate (as end-diastolic volumes [EDV]/s) and percent stroke volume filled at first third of diastole and at the end of the rapid filling period were determined. Group 2 had a reduced peak filling rate (2.58 +/- 0.65 EDV/s, p less than 0.05) compared with group 1 (3.11 +/- 0.65 EDV/s). Similarly, the percent stroke volume filled at the end of the rapid filling period was reduced in group 2. Group 3 patients had a lower peak filling rate and mean filling rate than group 1 patients. However, percent stroke volume filled at first third of diastole and percent stroke volume filled at the end of the rapid filling period were greater in group 3 than in group 1. Two distinct patterns of diastolic filling were noted in patients with AS. Group 2 patients had reduced peak filling rates with less diastolic filling during the rapid filling period. Although the peak filling rate was reduced in group 3, more complete filling occurred during the rapid filling period.     

Sinus of valsalva aneurysm with congenital aortic stenosis and aortic coarctation

A 52-year-old female was diagnosed with the extremely rare combination of sinus of Valsalva aneurysm, congenital unicuspid aortic valve stenosis, and coarctation of the aorta. She was successfully treated in a two-stage operation. The first operation consisted of a bypass graft to correct the coarctation. The second, performed 31 days later, included aortic valve replacement, patch closure of the aneurysm, and translocation of the right coronary artery.     

Left ventricular wall stress in compensated aortic stenosis in children

It is known that children with aortic stenosis (AS) frequently have supernormal indexes of left ventricular (LV) pump function and remain compensated for many years. Factors causing this increase in pump performance have not been elucidated. A study was done on LV mechanics in 11 children with AS (aortic valve area 0.5 +/- 0.3 cm2/m2) and 10 normal subjects. The ejection fraction in the AS group (0.88 +/- 0.08) was significantly higher than in normal subjects (0.64 +/- 0.08, p less than 0.001). The mean velocity of fiber shortening was also higher in AS patients (1.80 +/- 0.35 circ/s) than in normal subjects (1.22 +/- 0.21 circ/s, p less than 0.001). The end-systolic volume index in patients with AS (9 +/- 8 ml/m2) was much lower than in normal subjects (27 +/- 8 ml/m2). LV mass in patients with AS was 180 +/- 58 g/m2 compared with 96 +/- 9 in normal subjects. LV wall stress was reduced throughout the cardiac cycle in patients with AS. Peak stress in patients with AS was 238 +/- 51 dynes/cm2 X 10(3) versus 439 +/- 85 in normal subjects. The end-systolic stress-end-systolic volume index ratio, an indicator of contractile state, was not elevated in patients with AS. It is suggested that diminished wall stress in concert with normal contractile function permits the supernormal pump function seen at rest in children with AS.     

不同手术方法治疗先天性主动脉瓣上狭窄的临床研究

目的：探讨先天性主动脉瓣上狭窄外科纠治的手术适应证选择、不同手术方法的近、中期疗效。方法：回顾性分析西京医院2005年2月至2020年2月先天性主动脉瓣上狭窄外科治疗病例27例,年龄1.4～13.5y（4.1±3.2）,合并染色体异常（Williams综合征）19例（70.4%）。术前主动脉瓣上峰值压差45～98(72.3±13.4)mmHg,其中。其中局限型24例(88.9%),弥漫型3例(11.1%)。主动脉瓣不同程度增厚24例,瓣窦发育不均衡9例,合并少量或微量主动脉瓣反流5例,无主动脉瓣狭窄。合并左、右肺动脉分支狭窄9例。手术均在中低温体外循环下实施,采用单片（McGoon）法7例(25.9%),双片(Doty)法11例(40.7%),三片法9例(33.3%),其中Brom法4例,Myers法5例。肺动脉狭窄同期补片加宽纠治。结果：全组无住院死亡。术后主动脉瓣上峰值压差14～54(21.6±8.4)mmHg,其中单片组20～54(30.6±11.9)mmHg,双片组14～23(19.3±2.9)mmHg,三片组14～22(17.6±2.5)mmHg,单片组与其他两组差异明显（p?0.01）,双片组与三片组无明显差异（p?0.05）。主动脉瓣少量反流2例,微量6例。随访21例,失访6例,随访3月～11年（平均3.7年）,主动脉瓣上峰值压差17～46(24.1±7.4)mmHg,其中单片组26～46(33.4±10.5)mmHg,双片组19～31(22.3±2.7)mmHg,三片组17～26(19.1±2.1)mmHg,单片组与其他两组差异明显（p?0.01）,双片组与三片组无明显差异（p?0.05）。无远期死亡和再次手术干预。主动脉瓣少量反流4例,微量7例。结论：手术纠治先天性主动脉瓣上狭窄是安全的,近、远期疗效满意。双片和三片法手术疗效无明显差异,但均优于单片法,应作为首选手术方法。     

Left ventricular aneurysm in a five-week-old child

Ventricular aneurysms in the pediatric population are rare and no standardized method of treatment exists. We present a case of left ventricular (LV) aneurysm in an infant, 5 weeks after a balloon aortic valvuloplasty for congenital aortic stenosis.     

Giant dissecting aortic aneurysm with concealed perforation in an 81-year-old female

This paper describes the history of an 81-year-old female suffering from a giant dissecting aortic aneurysm with concealed perforation within the thorax. The patient had suffered from arterial hypertension for about 10 years and had been treated with thiazide. Nine months prior to admission the patient was in a state of collapse, and ultrasound examination revealed an intra-abdominal aortic aneurysm. At this time thoracic x-ray showed aortic sclerosis and elongation of the aorta but no signs of aneurysm formation. After this episode the patient was symptom-free for the next 9 months. Following a further syncopal attack with severe thoracic pain, the patient was hospitalized at the intensive care unit. Both in thoracic x-ray and computed tomography of the thorax, a pronounced dissecting aortic aneurysm with perforation of the thoracic aorta into the mediastinum could be established. Because of the patient's poor general condition and advanced age, as well as far reaching pathological findings, surgery was not advised by either the heart and vascular surgeon or the anesthetist. Following 1 week's intensive therapy, the patient's general condition improved greatly, with stabilization of thoracic pain, blood pressure, and respiratory action. On the other hand, thoracic x-ray, computed tomography, and magnetic resonance imaging produced a distinct progression of the aneurysm with consequent mild displacement of mediastinum and left lung. Laboratory examinations for syphilis showed no evidence of that disease. After further improvement the patient was discharged 4 weeks after admission and has been symptom-free for 6 months in spite of the extensive pathological findings described herein. This case demonstrates the efficacy of conventional therapy when surgery is not recommended.