Tetralogy of Fallot

Hearts with Fallot's tetrad from 33 children dying at the age from 14 days to 8 years were studied. Analysis of the morphological features of the defect revealed its typical anatomic signs: anteroleft shift of conic septum (oblique orientation); reorientation of the structures of inflow and outflow parts of the right ventricle infundibular pulmonary stenosis the occurrence of which may be due to all the structures and formations participating in the formation of the outflow part of the right ventricle. Four main types of stenoses were established. The most frequent cause of maximal stenosis of the blood flow through the right ventricle was demarcating muscle ring; a nonrestrictive defect of the ventricular septum (DVS). Three types of DVS in Fallot's tetrad were found: perimembranous, muscular, subarterial; dextroposition of the aorta, and right ventricular hypertrophy.     

The position of the left and right ventricular outlets during septation

Summary A comparative study was made of the relative position of the outflow tracts of chicken and rat hearts with respect to the ventricles during septation. For this purpose the position of the left and right ventricular outlet including the aortic and pulmonary valve primordia and the left and right ventricle were established with respect to the midsagittal plane of the embryo, using reconstructions of serial sections of chicken (stage 28–30) and rat (stage 28–30) embryos. In the chicken embryo no rotation of the outflow tract occurs, i.e. the position of the aortic and pulmonary valve primordia with respect to the left and right ventricle remains the same. In the rat embryo a clockwise rotation of the aortic and pulmonary valve primordia with respect to the ventricles does occur. This is in fact a detorsion. The left and right ventricle and the left ventricular outlet do not show change in position with regard to the midsagittal plane. The left ventricular outlet always straddles the interventricular septum, both lying in the midsagittal plane. These interspecies differences in the degree of detorsion of the outflow channels before septation may explain the differences in the extent of the region of contact between the endocardial outflow tract ridges.     

Surgical repair of congenital supravalvular aortic stenosis in adult

Supravalvular aortic stenosis is a rare congenital cardiac anomaly occurring mainly as a part of Williams-Beuren syndrome. Aortic narrowing above the level of the aortic valve causes obstruction of the left ventricular outflow tract, and a pressure gradient between the left ventricle and the aorta causes left ventricle hypertrophy. We report here a case of a 22-year-old man who underwent extended patch aortoplasty because of supravalvular aortic stenosis accompanying Williams-Beuren syndrome. He was in New York Heart Association functional class III with localized hourglass type supravalvular aortic stenosis. Related to arterial hypertension he was in a cardiac decompensation. Mean pressure gradient was 73 mm Hg and maximum gradient 104 mm Hg. Electrocardiography indicated left ventricle hypertrophy, which was also seen in x-ray, as heart enlargement. We successfully treated this patient with extended patch aortoplasty and immediate postoperative echocardiography showed reduction of gradient. Good surgical outcome of congenital supravalvular aortic stenosis in adults can be achieved with this treatment. This technique provides symmetric reconstruction of the aorta with good postoperative results and no gradient across aortic valve and aortic valve insufficiency remains, providing excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence.     

室间隔射频消融术在梗阻性肥厚型心肌病中的应用

目的探讨室间隔射频消融术(PIMSRA)治疗梗阻性肥厚型心肌病(HOCM)的临床效果。方法收集2019年6~8月于我院心血管内科行PIMSRA的7例HOCM患者的临床资料,回顾分析该治疗方法的手术要点、术后管理重点和随访结果。结果7例患者手术均获成功,术中、术后未出现严重并发症,术后左心室流出道压力阶差下降明显,超声显示二尖瓣前叶收缩期前向运动现象明显减轻。出院后15 d、2个月、3个月随访,7例患者生存良好,疾病症状均缓解,心功能分级均有改善。结论PIMSRA治疗HOCM安全可靠,术后即刻可降低左室流出道压力阶差,改善患者临床症状。     

External carotid pulse recordings in hypertrophic obstructive cardiomyopathy

External carotid pulse tracings were examined in 15 patients with hypertrophic obstructive cardiomyopathy (HOCM), the diagnosis being confirmed by catheterization of the left heart. Of 12 patients with intraventricular gradients at rest, 9 had a typical bifid pulse with midsystolic dipping. In one patient without a gradient at rest, midsystolic dipping occurred only in beats following extrasystoles (Brockenbrough phenomenon). The upstroke of the pulse wave was rapid in all the patients, a finding that distinguishes them from patients with valvular aortic stenosis. There was a correlation between the length of the left ventricular ejection time and the intraventricular gradient (r = 0.71) but as more than half the patients had normal or shortened ejection times, the diagnosis of HOCM cannot be based on measurements of this parameter. It is concluded that carotid pulse registrations are of considerable diagnostic value in patients suspected of having HOCM. As the pulse changes are correlated to the degree of left ventricular outflow obstruction, it is suggested that repeated pulse tracings may be used as a means for controlling the degree of obstruction once the diagnosis has been established in the individual patient.     

Pacemaker lead endocarditis caused by Achromobacter xylosoxidans

We report the case of a 35-yr-old patient who presented with high fever and chills. He had undergone a patch closure of the ventricular septal defect 18 yr before. One year later, a VVI pacemaker was implanted via the right subclavian vein because of complete heart block. Nine years after that, a new VVI pacemaker with another right ventricular electrode was inserted controlaterally and the old pacing lead was abandoned. Trans-thoracic and trans-esophageal echocardiogram identified the pacemaker lead in the right ventricle (RV) attaching hyperechoic materials and also a fluttering round hyperechoic mass with a stalk in the RV outflow tract. Cultures in blood and pus from pacemaker lead grew Achromobacter xylosoxidans. A diagnosis of pacemaker lead endocarditis due to Achromobacter xylosoxidans was made. In this regards, the best treatment is an immediate removal of the entire pacing system and antimicrobial therapy.     

Characterization of conotruncal malformations following ablation of "cardiac" neural crest

Neural crest cells from the cranial region of the neural fold populate the outflow tract of the developing chick heart. Removal of this region of premigratory neural crest has been shown previously to result in a high percentage of conotruncal malformations. The present study was undertaken to define more precisely the regions of premigratory neural crest which are needed for normal conotruncal development. Various regions and lengths of premigratory cranial neural crest were ablated using microcautery. Three defects in conotruncal development were significantly correlated with the neural crest ablation. These were high ventricular septal defect, single outflow vessel originating from the right ventricle, and single outflow vessel overriding the ventricular septum.     

Infective endocarditis complicating hypertrophic obstructive cardiomyopathy: an unusual mural pattern

A patient with hypertrophic obstructive cardiomyopathy (HOCM) and an unusual right ventricular mural pattern of endocarditis that clinically mimicked a neoplasm is presented. To our knowledge, this is the first report of right ventricular mural endocarditis complicating HOCM.     

缺氧性肺动脉高压大鼠右心室重构

摘要目的：研究缺氧性肺动脉高压大鼠右心室重构情况。方法：常压间断缺氧法复制缺氧性肺动脉高压大鼠模型，采用右心导管法测定平均肺动脉压力，通过测量右心室流入及流出道长度、左心室壁和右心室壁厚度、右心室和左心室 室间隔重量对其右心室重构情况进行定性研究。结果：缺氧14d后大鼠平均肺动脉压力显著升高，右心室流出道长度及右心室肥大指数显著增加，缺氧21d后右心室游离壁重量显著增加；右心室流人道长度及左、右心室壁厚度与对照组无统计学差异。结论：缺氧性肺动脉高压大鼠右心室早期表现为离心性肥大。     

Myocardial volume and organization are changed by failure of addition of secondary heart field myocardium to the cardiac outflow tract.

Cardiac neural crest ablation results in primary myocardial dysfunction and failure of the secondary heart field to add the definitive myocardium to the cardiac outflow tract. The current study was undertaken to understand the changes in myocardial characteristics in the heart tube, including volume, proliferation, and cell size when the myocardium from the secondary heart field fails to be added to the primary heart tube. We used magnetic resonance and confocal microscopy to determine that the volume of myocardium in the looped heart was dramatically reduced and the compact layer of myocardium was thinner after neural crest ablation, especially in the outflow tract and ventricular regions. Proliferation measured by 5-bromo-2'-deoxyuridine incorporation was elevated at only one stage during looping, cell death was normal and myocardial cell size was increased. Taken together, these results indicate that there are fewer myocytes in the heart. By incubation day 8 when the heart would have normally completed septation, the anterior (ventral) wall of the right ventricle and right ventricular outflow tract was significantly thinner in the neural crest-ablated embryos than normal, but the thickness of the compact myocardium was normal in all other regions of the heart. The decreased volume and number of myocardial cells in the heart tube after neural crest ablation most likely reflects the amount of myocardium added by the secondary heart field.     

Pulmonary artery stenosis with an intact interventricular septum

15 hearts with pulmonary artery stenosis with an intact interventricular septum (PAS with IIVS) were studied morphologically and morphometrically. The results were evaluated separately according to the type of the defect: with a noncomplicated valvular stenosis of pulmonary artery (PA); with a combined valvular and infundibular stenosis of PA. Every of these types of PAS with IIVS has characteristic features of morphological structure and all possess common anatomical signs: constriction at one of the levels of circulation from the right ventricle to the lesser circulation, normal geometric structure of the cone and myocardial hypertrophy of the right ventricle. Leading cause of the obstruction of the right ventricle effluent part, regardless of the PAS with IIVS type, is a hypertrophy of the supraventricular crest and its parietal projection. As distinct from the defects on the conus-truncus, complex of PAS with IIVS is characterized by a normal spatial orientation of the supraventricular crest.     

Small left atrium: an adjunctive sign of hemodynamically compromised massive pulmonary embolism

Pulmonary embolism (PE) is a common disease with a high mortality rate due to right ventricular dysfunction and underfilling of the left ventricle. We present a case of a 33-year-old man with hemodynamically compromised massive PE. His left atrium was collapsed with marked dilatation of the right atrium and ventricle on multi-detector-row CT scans. The patient was treated with an intracatheter injection of a mutant tissue-type plasminogen activator and subsequently showed clinical and radiological improvements. The small left atrial size in combination with a right ventricular pressure overload was considered to be an adjunctive sign of hemodynamically compromised massive PE.     

Characterization of conotruncal malformations following ablation of “cardiac” neural crest

Neural crest cells from the cranial region of the neural fold populate the outflow tract of the developing chick heart. Removal of this region of premigratory neural crest has been shown previously to result in a high percentage of conotruncal malformations. The present study was undertaken to define more precisely the regions of premigratory neural crest which are needed for normal conotruncal development. Various regions and lengths of premigratory cranial neural crest were ablated using microcautery. Three defects in conotruncal development were significantly correlated with the neural crest ablation. These were high venticular septal defect, single outflow vessel originating from the right ventricle, and single outflow vessel overriding the ventricular septum.     

镜面右位心的诊断和外科治疗——附11例临床分析

目的：分析镜面有位心病理解剖特点，探讨其临床诊断及外科治疗方法。方法：总结11例镜面右位心临床资料，对其主要的合并畸形、诊断方法和其中6例外科治疗的效果进行比较分析。结果：本组患者包括右心室双出口4例，单心室合并单心房2例，法乐四联征3例，完全性房室间隔缺损2例。合并畸形包括双上腔静脉3例，房间隔缺损3例，部分性肺静脉异位引流2例，三房心1例，动脉导管未闭1例。10例患者合并肺动脉狭窄或右室流出道狭窄，1例重度肺动脉高压。主要根据胸部X线片，超声心动图（UCG）及心导管明确诊断。行右心室双出口矫正术2例，法乐四联征根治术3例，右心室流出道疏通1例。4例存活，2例死亡。结论：（1）镜面右位心多存在复杂心内畸形。（2）UCG仍然是最基本有效的诊断方法，心导管检查是明确诊断的必要辅助手段。（3）外科治疗必须严格把握手术指征。     

Spectrum of looping disturbances in stage 34 chicken hearts after retinoic acid treatment

Background: In a recently developed chick model the teratogen retinoic acid has appeared to induce a spectrum of double outlet right ventricle, which needs further detailed evaluation. It is known that retionic acid is able to induce cardiac malformations. Although the exact mechanism is not known, an interaction with neural crest cell function is thought to exist. Methods: After treatment with 1 μg all-trans retinoic acid at Hamburger and Hamilton stage 15 and reincubation until stage 34 of development 41 chicken embryos were evaluated macroscopically and microscopically, supported by graphic reconstructions. These retinoic acid treated embryos were compared with a control group (n = 8). Results: The retinoic acid treated embryos could be divided in three groups. Group 1 (23/41) had an intact septum, group 2 (11/41) had an isolated ventricular septal defect (VSD), and group 3 (7/41) had a double outlet right ventricle (DORV). Besides, in the group with an intact septum 11 hearts showed an abnormal course of the subaortic outflow tract. In the group with DORV a straddling tricuspid orifice (7/8) and a double inlet left ventricle (1/8) could be distinguished. Considering the external contour, the hearts in the DORV group all showed a dextroposed arterial pole. Malformed pharyngeal arch arteries were found in all three groups (11/41) and with a great diversity. Conclusions: The present cardiac malformations in the chicken as a result of retinoic acid treatment are part of a continuous spectrum, varying from hearts with an intact ventricular septum and a normal course of the subaortic outflow tract to a double outlet right ventricle with a straddling tricuspid orifice or even a double inlet left ventricle. A remarkable observation in this spectrum concerns the correlation of malformations of the inflow and outflow tracts, which is explained as a cardiac looping disturbance. The disturbance of the looping process seems to lead to malalignment of septal components, although, in the chick, retinoic acid does not in general interfere with the formation of these septal components themselves. © 1995 Wiley-Liss, Inc.     

Intramitochondrial Glycogen in Hypertrophied Infundibular Muscle of Patients with Congenital Heart Diseases

A small population of glycogen-containing mitochondria was found in operatively resected myocardium in 8 of 11 patients with various types of congenital heart diseases associated with right ventricular hypertrophy and muscular (infundibular) obstruction to right ventricular outflow. The presence of intramitochondrial glycogen correlated with the degree of peripheral arterial oxygen desaturation but not with the severity of hemodynamic alterations or with the ages of the patients. The occurrence of intramitochondrial glycogen in myocardium in other conditions is reviewed, and its possible pathogenesis is discussed.     

Radiofrequency ablation can reverse the structural remodeling caused by frequent premature ventricular contractions originating from the right ventricular outflow tract even in a “normal heart”

OBJECTIVE:

The aim of this study was to evaluate whether frequent premature ventricular contractions originating from the right ventricular outflow tract remodel the cardiac structure and function in patients with a “seemingly normal heart” and whether radiofrequency ablation can reverse this remodeling.

METHODS:

Sixty-eight patients with idiopathic frequent premature ventricular contractions originating from the right ventricular outflow tract and normal heart structure and function were enrolled in this study. The patients were divided into three groups according to the therapeutic method: radiofrequency ablation group (24 cases), anti-arrhythmia drug group (26 cases), and control group (18 cases without any treatment). Clinical Registration number: ChiCTR-ONRC-12002834

RESULTS:

The basic patient characteristics were comparable between the three groups, except for the premature ventricular contraction rate, which was significantly lower in the control group. After six months of follow up, the premature ventricular contraction rate was significantly reduced in the radiofrequency ablation group, which was accompanied by a significant decrease in the following cardiac cavity inner diameters, as determined by echocardiography: right atrium (33.33±3.78 vs. 30.05±2.60 mm, p = 0.001), right ventricle (23.24±2.40 vs. 21.05±2.16 mm, p = 0.020), and left ventricle (44.76±4.33 vs. 41.71±3.44 mm, p = 0.025). These results were similar in the anti-arrhythmia drug group, although this group exhibited a smaller extent of change (right atrium: 33.94±3.25 vs. 31.27±3.11 mm, p = 0.024; right ventricle: 22.97±3.09 vs. 21.64±2.33 mm, p = 0.049; left ventricle: 45.92±6.38 vs. 43.84±5.67 mm, p = 0.039), but not in the control group (p>0.05). There was a tendency toward improvement in the cardiac functions in both the radiofrequency ablation and anti-arrhythmia drug groups. However, these differences were not statistically significant (p>0.05).

CONCLUSIONS:

These results indicate that radiofrequency ablation can potentially reverse the cardiac remodeling caused by frequent premature ventricular contractions even in structurally normal hearts and that frequent premature ventricular contractions should be abated even in structurally normal hearts.     

Exophytic cavernous hemangioma arising from the right ventricle: Report of a rare case

Cardiac hemangioma is relatively rare, accounting for approximately 1–3% of all primary heart tumors. This benign tumor may be an incidental lesion, but can also cause arrhythmias, pericardial effusion, congestive heart failure or outflow obstruction. We report a rare case with exophytic cardiac hemangioma arising from the right ventricle. Echocardiography showed an approximately 40 mm round protruding mass on the anterior wall of the right ventricle. Cardiovascular magnetic resonance demonstrated isointense and hyperintense signals on T1- and T2-weighted images, respectively. These imaging studies suggested a pericardial cyst. Perioperative findings indicated a globular, exophytic mass, vascular in nature, arising from the right ventricle. The lesion was resected directly, and the space left by defect in the right ventricular wall was covered with a bovine pericardial patch. Cardiac hemangiomas are generally endoluminal tumors, but we must keep in mind that the differential diagnoses include various pericardial lesions by medical images.     

右室双腔心的外科治疗

目的 总结右室双腔心的解剖诊断和外科治疗经验.方法 回顾性分析2001年9月-2014年6月手术治疗76例右室双腔心患者的临床资料.所有患者均在低温、浅低温体外循环下施行根治手术.58例采用三尖瓣径路＋右室流出道径路（A组）,18例采用单纯三尖瓣径路或合并肺动脉径路（B组）.比较两组术中、术后情况.结果 76例患者均顺利完成手术.术中无停机困难、大出血等情况发生.A组患者体外循环时间、术后24 h平均多巴胺用量、辅助通气时间、术后24 h引流量和右束支传导阻滞发生率均高于B组,差异均有统计学意义（P值均＜0.01）;而疏通术后收缩压差、主动脉阻断时间与B组比较,差异均无统计学意义（P值均＞0.05）.全组无一例死亡.术后并发低心排血量综合征5例,严重心律失常6例,呼吸功能衰竭1例,经对症处理后均治愈.76例均获随访,随访时间3个月～13年,平均4.26年.所有患者无死亡,心功能恢复满意.结论 熟练掌握右室双腔心的病理解剖、正确有效地疏通右心室腔是手术成功的关键;通过三尖瓣或右心室流出道均可解除右室双腔心梗阻,但三尖瓣径路可获得更好的近远期效果.     

A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction

Pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.