Value of the echocardiographic findings of arrhythmogenic right ventricular dysplasia with left ventricular involvement in a child

A 9-year-old girl was admitted because she had had palpitations for 2 years. Her electrocardiogram revealed multifocal ectopic beats with left bundle branch morphology, sinoatrial block, and first- and second-degree atrioventricular blocks. Cross-sectional echocardiography demonstrated an enlarged left ventricle and filling defects and sacculations of the posterior left ventricular wall, apex, and septum. The diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) was confirmed by endomyocardial biopsy and thallium 201 scintigraphy. We present this case because of the rarity of left ventricular involvement associated with ARVD. We also emphasize the value of echocardiography in the early diagnosis of ARVD.     

Atrioventricular septal defect: Quantitative anatomy of the right ventricle

Summary In 19 human fetal and newborn hearts with atrioventricular septal defect (AVSD), not associated with other anomalies, the two ventricles were studied morphometrically. A total of 17 different parameters were studied: nine in the right ventricle and eight in the left. In the right ventricle we analyzed ventricular wall thickness, length of right ventricular inflow and outflow tracts, and volume of right ventricular inflow and outflow tracts. The data for these parameters were compared with the patterns of normality published previously, and the volumetric data were compared with patterns of normality published previously by us. The ventricular inflow tract was shorter than the outflow tract, the difference being especially significant in the left ventricle. The length of the diaphragmatic wall of the heart in both the right and left ventricle was equal to the sum of the length of the inflow tract and the thickness of the ventricular wall at the apex.     

Diffuse myocardial fibrosis following tetralogy of Fallot repair: a T1 mapping cardiac magnetic resonance study

Background

Adverse ventricular remodeling after tetralogy of Fallot (TOF) repair is associated with diffuse myocardial fibrosis.

Objective

The goal of this study was to measure post-contrast myocardial T1 in pediatric patients after TOF repair as surrogates of myocardial fibrosis.

Materials and methods

Children after TOF repair who underwent cardiac magnetic resonance imaging with T1 mapping using the modified look-locker inversion recovery (MOLLI) sequence were included. In addition to routine volumetric and flow data, we measured post-contrast T1 values of the basal interventricular septum, the left ventricular (LV) lateral wall, and the inferior and anterior walls of the right ventricle (RV). Results were compared to data from age-matched healthy controls.

Results

The scans of 18 children who had undergone TOF repair and 12 healthy children were included. Post-contrast T1 values of the left ventricular lateral wall (443?±?54 vs. 510?±?77 ms, P?=?0.0168) and of the right ventricular anterior wall (333?±?62 vs. 392?±?72 ms, P?=?0.0423) were significantly shorter in children with TOF repair than in controls, suggesting a higher degree of fibrosis. In children with TOF repair, but not in controls, post-contrast T1 values were shorter in the right ventricle than the left ventricle and shorter in the anterior wall of the right ventricle than in the inferior segments. In the TOF group, post-contrast T1 values of the RV anterior wall correlated with the RV end-systolic volume indexed to body surface area (r?=?0.54; r²?=?0.30; P?=?0.0238).

Conclusion

In children who underwent tetralogy of Fallot repair the myocardium of both ventricles appears to bear an abnormally high fibrosis burden.     

Left ventricular thrombus in a 2-year-old boy with cardiomyopathy: Lysis with recombinant tissue-type plasminogen activator

A 2-year-old boy with cardiomyopathy and clinical signs of cardiac failure presented with an echodense structure in the left ventricle. This structure was seen from different echocardiographic views adjacent to a hypokinetic area of the apex and lateral free wall. It was different in texture and motion from the underlying myocardium and thus met the diagnostic criteria of a left ventricular thrombus. This thrombus protruded into the cavum and was partly mobile. In view of a high embolic risk, thrombolytic therapy with recombinant tissue plasminogen activator was started. The thrombus resolved within 72 h without any embolic or bleeding complications. No recurrence of the thrombus was observed during a 3-month follow up period.     

Echocardiogram in mitral-aortic atresia: False identification of the ventricular septum and left ventricle

Summary M-mode echocardiograms were correlated with cardiac anatomy in six newborn infants who had autopsy-confirmed mitral-aortic atresia. Echoes from the mitral valve or the aortic valve were not identified. An echo resembling that from the ventricular septum was recorded posterior to the tricuspid valve in all six infants. This echo was separated from the posterior ventricular wall echo by a space that varied in width from 3 to 8 mm. In two infants, the echo was continuous with that from the posterior aspect of the aortic root area and in all six infants displayed an anterior systolic motion pattern. Autopsy demonstrated that in every case the left ventricle was minute and was located superiorly in the wall of the right ventricle; in life, the transducer beam would not have traversed the left ventricle. The right ventricle was enlarged in every case and had prominent trabeculations of the septal band that created large anatomic spaces within the right ventricle. This study suggests that echoes previously thought to represent the left ventricle and ventricular septum in infants with mitral-aortic atresia do not represent these structures and are possibly created by the unusual right ventricular anatomy. Recognition of this false-positive finding may be useful in the interpretation of echocardiograms of other lesions in which there are abnormalities of the aortic or mitral valve, especially if there is a dominant right ventricle. Supported by grant HL-07605 from the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland     

Single papillary muscle of the left ventricle associated with persistent common atrioventricular canal: Variant of parachute mitral valve

Summary Six cases of single papillary muscle of the left ventricle in persistent common atrioventricular canal (AVC) are described. Except for one case with double outlet right ventricle and AVC, in the other five cases AVC occurred with normally related great vessels. Down's syndrome was present in two cases. The mitral valvular condition in AVC is not the classic parachute mitral valve since the separation between the common anterior and the common posterior leaflet leaves a space (cleft) oriented toward the ventricular septum. This mitral valve in AVC may or may not be obstructive. The common anterior leaflet fixed to the ventricular septum may result in obstruction. Clinically, single left ventricular papillary muscle should be suspected if the AVC is associated with coarctation of the aorta or tubular hypoplasia of the aortic arch. This type of mitral valve in AVC may also be associated with double outlet right ventricle. Preoperative recognition of the single papillary muscle by echocardiogram or left ventriculogram will prevent postoperative mitral obstruction following closure of the mitral cleft.     

Normal pulmonary vascular resistance and left ventricular hypertrophy in young infants with bronchopulmonary dysplasia: an echocardiographic and pathologic study

To evaluate the cardiac anatomy and functional hemodynamics in young infants with chronic lung disease, nine patients, aged 2 to 7 months, with a clinical diagnosis of bronchopulmonary dysplasia (BPD) underwent echocardiographic examination. All infants required supplemental O2 (mean FIO2 35%) to maintain adequate systemic oxygenation (Pao2 greater than 50 mm Hg). None of the infants had evidence of a patent ductus arteriosus at the time of examination. Echocardiographic measurements of left and right ventricular systolic time intervals revealed normal systolic time interval ratios suggesting pulmonary vascular resistances. However, echocardiographic evidence of left ventricular hypertrophy was found in eight of the nine infants, while right ventricular anterior wall thickness and right ventricular diastolic dimensions were not increased. Two infants died; marked left ventricular hypertrophy was noted at the time of postmortem examination while the right ventricular wall thickness was normal. The findings of left ventricular hypertrophy led to a retrospective review of autopsy material of seven patients who died with BPD over the past year. In six of seven cases examined, left ventricular posterior wall thickening was noted (range 7 to 11 mm); while the right ventricular wall thickness was normal (range 2 to 5 mm). These data suggest that (1) as assessed by echocardiography, the pulmonary vascular resistance is not significantly elevated in young infants with BPD, and (2) a hypertrophic left ventricle evolves which may assume importance in the pathogenesis of pulmonary edema in BPD, though the precise etiology remains undetermined.     

Long-term survival of aortic atresia following biventricular corrective surgery

A female born with aortic atresia, large ventricular septal defect, normal mitral valve, and left ventricle is well at 21 years of age following biventricular repair. She had palliative surgery at 15 days and closure of ventricular septal defect with placement of a valved conduit from the left ventricular apex to descending aorta at 15 months. Conduit was replaced at 34 months and at 10 and 21.5 years of age.     

Value of the new Doppler-derived myocardial performance index for the evaluation of right and left ventricular function following repair of tetralogy of fallot

The systolic and diastolic function in both ventricles may be altered even after successful corrective surgery of tetralogy of Fallot. The aim of this study was to assess the combined diastolic and systolic function of both ventricles using the Doppler-derived myocardial performance index (MPI) in patients with operated tetralogy of Fallot (TOF). We performed a prospective analysis of 51 patients following corrective surgery of TOF: 21 had a subannular patch, 20 had a homograft implantation at initial operation, and 10 were reoperated with secondary homograft implantation. Patients were examined with Doppler echocardiography, and the MPI, which incorporates ejection and isovolumetric relaxation and contraction times and is an index of global ventricular function, was calculated 10.2 +/- 8.0 (0.89-36) years after surgery. In 86.4% of the examined patients the right ventricular isovolumetric relaxation time was shortened compared to the normal published range or even did not exist (negative value) (p <0.01). The right ventricular MPI was paradoxically below the normal published range in 76.5% of the examined patients. The left ventricle global function was impaired in 23.5% of the examined patients, mainly due to altered systolic function with a prolonged left ventricular isovolumetric contraction time. The z score of the comparison between patients' left ventricular isovolumetric contraction time and the normal published values was 3.03. Patients with severe pulmonary regurgitation also had a prolongation of the isovolumetric relaxation time compared to patients with mild to moderate pulmonary regurgitation. The noncompliant right ventricle may shorten the right ventricular isovolumetric relaxation time, resulting in a paradoxically low right MPI. This may reduce the sensitivity of the index in recognizing patients with right ventricular dysfunction following corrective surgery of TOF. Additional diastolic impairment occurs in patients with right ventricular volume overload.     

The Usefulness of Doppler Echocardiography for the Evaluation of Hemodynamics in Tetralogy of Fallot: Comparison of the Patients with Pre- and Post-Corrective Surgery and Control Healthy Children

Twodimensional Doppler echocardiographic examinations were performed in 61 patients (39 boys and 22 girls) with tetralogy of Fallot (TOF). The patients were examined at the Department of Pediatrics, University of Tokyo, and at the Department of Pediatrics, Ohta General Hospital, in Koriyama city, Fukushima Prefecture. One hundred and eighteen healthy children (59 boys and 59 girls) were also examined at the Waseda housing complex in Misato city, Saitama Prefecture. By short axis view of aorta, samples were collected from both the right ventricular outflow tract below the pulmonary valve and in the main pulmonary artery. On the other hand, the apex four chamber view was used to collect samples at the right and left atrial outflows. Right ventricular systolic time intervals were measured both at the main pulmonary artery (PA) and at the outflow tract below the pulmonary valve (RVO). The right ventricular systolic pre-ejection period (PAPEP) and acceleration time (PAACT), measured at the supra-valvular site, were shorter before surgery as compared to after surgery and normals. The ratio of right ventricular pre-ejection period to ejection time (PAPEP/ET) and the ratio of acceleration time to ejection time (PAACT/ET) were both significantly lower before surgery compared to after surgery and controls. The mean main pulmonary artery peak velocity before surgery was 369 cm/sec and the peak pressure gradient calculated with the modified Bernoulli formula was 55 mmHg. Before surgery, peak velocity of right ventricular rapid filling (R-wave) was low, while peak velocity of atrial contraction (A-wave) was high. After surgery, however, R-wave increased and A-wave decreased in velocity. The peak velocity of left ventricular filling R-wave was also low before surgery and increased after surgery, while peak velocity of left atrial contraction A-wave was increased before surgery and decreased to almost normal values after surgery. The left ventricular A/R ratio before surgery was also high as a result of low R-wave velocity. The measured time interval between aortic valve closing sound and left ventricular rapid filling wave (IIA-R time), and the measured time interval between pulmo nary valve closing sound and ventricular filling wave (IIP-R time) were both longer before surgery than those after surgery and controls. Consequently, the measured time intervals between rapid ventricular filling flow wave and atrial contraction wave (R-A time) for both right and left atrial were shorter before surgery than after surgery and controls. A new method for estimation of pulmonary-to-systemic blood flow ratio (Qp/Qs) was successfully applied to TOF, and a good correlation was obtained between Qp/Qs determined by this method and that by the Fick method.     

Morphologic Features of the Hypoplastic Left Heart Syndrome-A Reappraisal

The report describes the morphologic features of 102 specimens with hypoplasia of the chambers of the left heart, all with either a concordant atrioventricular connection (66 cases) or absence of the left atrioventricular connection, the right atrium being connected to a dominant right ventricle (36 cases). Either the aorta was connected to the left ventricle, having a patent valve in 23 cases and an imperforate valve in 5, or else the aortic trunk was atretic (74 cases). It was not possible to determine with certainty the ventricular origin of the aorta when its trunk was atretic, but in all hearts but 1 it seemed to be connected to the left ventricle. The atrial septum was intact in 11 hearts and grossly restrictive in a further 8. Deviation of the atrial septum relative to the infolded atrial roof (septum secundum) was seen in 32 specimens. The ventricular septum was intact in 88 hearts. The length of the left ventricle compared to the right was greatest in the 14 hearts with a ventricular septal defect. The left ventricular length was also significantly greater in hearts with a patent aortic valve. The state of the atrial septum, in contrast, bore no relationship to the dimensions of the left ventricle. Aortic coarctation was found in 44 of the hearts, and in all but 2 the shelf lesion was preductal in location. Coarctation was significantly more frequent in those hearts in which the ascending aorta was less than 3 mm in diameter. The anatomic findings are discussed in relation to recent advances in the surgical palliation of these malformations.     

Morphologic features of the hypoplastic left heart syndrome--a reappraisal

The report describes the morphologic features of 102 specimens with hypoplasia of the chambers of the left heart, all with either a concordant atrioventricular connection (66 cases) or absence of the left atrioventricular connection, the right atrium being connected to a dominant right ventricle (36 cases). Either the aorta was connected to the left ventricle, having a patent valve in 23 cases and an imperforate valve in 5, or else the aortic trunk was atretic (74 cases). It was not possible to determine with certainty the ventricular origin of the aorta when its trunk was atretic, but in all hearts but 1 it seemed to be connected to the left ventricle. The atrial septum was intact in 11 hearts and grossly restrictive in a further 8. Deviation of the atrial septum relative to the infolded atrial roof (septum secundum) was seen in 32 specimens. The ventricular septum was intact in 88 hearts. The length of the left ventricle compared to the right was greatest in the 14 hearts with a ventricular septal defect. The left ventricular length was also significantly greater in hearts with a patent aortic valve. The state of the atrial septum, in contrast, bore no relationship to the dimensions of the left ventricle. Aortic coarctation was found in 44 of the hearts, and in all but 2 the shelf lesion was preductal in location. Coarctation was significantly more frequent in those hearts in which the ascending aorta was less than 3 mm in diameter. The anatomic findings are discussed in relation to recent advances in the surgical palliation of these malformations.     

Double-Orifice Mitral Valve Associated with Noncompaction of Left Ventricular Myocardium

Double-orifice mitral valve (DOMV) is a rare anomaly commonly associated with other congenital heart diseases. We present two patients with DOMV and noncompaction of the left ventricular myocardium (NLVM). Case 1 was a 5-year-old male diagnosed with dilated cardiomyopathy. His echocardiogram showed thin myocardium with dilatation at the basal of the left ventricle, thick noncompacted myocardium around the apex of the left ventricle, and DOMV (complete bridge type) with mild mitral regurgitation. Case 2 was an 11-year-old male diagnosed with complete atrioventricular block. His echocardiogram showed thick noncompacted myocardium with mild hypokinesis from the posterior to lateral wall and DOMV (complete bridge type) with mild mitral regurgitation. DOMV is commonly associated with congenital anomaly and always has an abnormal subvalvar apparatus. The mitral valve and its apparatus embryologically originate from the endomyocardium, which is thought to be the origin of noncompacted myocardium. We speculate that patients with DOMV may have NLVM.     

室间隔缺损修补术后少见并发症的超声心动图诊断病例系列报告

目的： 探讨室间隔缺损(VSD)修补术后少见并发症的超声心动图诊断要点。 方法：通过查询北京安贞医院（我院）超声影像报告系统及病案管理系统获取病例信息,纳入2013年1月至2018年4月在我院诊断或随访的VSD修补术后少见并发症的连续病例,术后时间不限,使用Philips IE33彩色多普勒超声诊断仪,选择S5-1探头,频率1~5 MHz。VSD修补术后少见并发症由我院经验丰富的医生确诊,并经团队医生共同确认。 结果：符合本文纳入标准的VSD修补术后少见并发症34例,男19例,女15例,年龄4个月至25岁,平均（4.7±5.9）岁。28例为术后左室-右房通道（LVRAC）形成,3例为术后主动脉瓣穿孔并主动脉瓣反流,1例为术后LVRAC形成合并主动脉瓣穿孔,2例为术后主动脉-左房通道形成。34例患者中6例接受外科手术矫治,5例LVRAC患者在随诊过程中自行愈合,其余23例患者继续随访观察中。LVRAC超声心动图表现：①心尖及剑突下四腔心切面显示三尖瓣隔瓣与二尖瓣前瓣之间回声中断,缺损位于三尖瓣环上方;②彩色多普勒显示从左室至右房的异常分流信号;③连续多普勒记录到收缩期高速射流频谱。主动脉瓣穿孔超声心动图表现：①左心室长轴及大动脉短轴切面显示无冠瓣或右冠瓣局部回声中断;②彩色多普勒显示源自瓣叶裂孔处的主动脉瓣反流。主动脉-左房通道超声心动图表现：①彩色多普勒显示从主动脉至左房的异常分流信号,左心室长轴、大动脉短轴及五腔心切面均可显示;②连续多普勒记录到双期连续性高速射流频谱。 结论：多切面扫查及密切结合血流频谱特点,可以明确VSD修补术后异常分流信号的诊断。     

The Relation Between Right Ventricular Function and Left Ventricular Morphology in Hypoplastic Left Heart Syndrome: Angiographic and Pathological Studies

Cases of hypoplastic left heart syndrome (HLHS) were studied angiographically in 18 patients and pathologically in 22 patients. They were divided into three subgroups according to the morphological features of the left heart: mitral atresia with aortic atresia (MA/AA), mitral stenosis with aortic atresia (MS/AA) and mitral stenosis with severe aortic stenosis (MS/AS). Patients with MS/AA had a significantly lower right ventricular end-diastolic volume index and more hypokinesis of the right ventricular posterior wall than those with MA/AA. MS/AA not only increased the thickness of the left ventricular posterior wall and interventricular septum but also increased endocardial thickness compared with MA/AA. Myocardial histology revealed more frequent abnormal findings such as myocardial necrosis, calcification and interstitial fibrosis in the mitral stenosis groups (i.e., MS/AA and MS/AS) than in MA/AA. Right ventricular function appeared to be greatly influenced by left heart structure. The presence of larger left ventricular muscle bulk and frequent myocardial damage seen in MS/AA seems disadvantageous to right ventricular end-diastolic volume and right ventricular wall motion.     

Congenital thrombotic occlusion of the ascending aorta and the aortic arch

Summary In a one-day-old male newborn with severe heart failure, the skin of the upper right thorax was pink, whereas the remaining areas were cyanotic. No peripheral pulses were palpable and the blood pressure could not be measured. On cardiac catheterization, systolic and diastolic pressures were elevated in the left ventricle (137/4/12 mmHg), but in the descending aorta, reached via a patent ductus arteriosus, the pressure was only 55/45 mmHg. O₂ saturation was 97% in the left ventricle and 67% in the descending aorta. Angiocardiography showed an extreme obstruction of the ascending aorta and the aortic arch. The infant died on the second day. Postmortem examination revealed a wall-adherent calcified thrombus that totally occluded the lumen. No etiologic explanation could be obtained from the histologic examination, anamnestic data, or clinical findings.     

A Single-Center Experience with Intracardiac Thrombosis in Children with Dilated Cardiomyopathy

Intracardiac thrombosis in patients with a dilated cardiomyopathy can be life threatening. This study investigated the incidence, risk factors, and outcome of intracardiac thrombosis in children with dilated cardiomyopathy. A retrospective review of the clinical records was performed in 83 children with dilated cardiomyopathy diagnosed from January 1995 to December 2008. Intracardiac thrombi were detected in 5 patients (6.0%). The intracardiac thrombi were found mainly in the left ventricle (n = 3). One patient had a thrombus in the left atrium at the time of diagnosis, and a right ventricular thrombus was found in 1 patient with unrepaired ventricular septal defect complicated by pulmonary hypertension. Intracardiac thrombosis developed during rapid deterioration of ventricular function, and all patients had a poor ejection fraction of the left ventricle. All patients were treated with heparinization, and thrombectomy was performed in 1 patient. Three patients achieved complete resolution of the thrombus without further embolic complications. Careful evaluation and aggressive anticoagulation are necessary for the prevention of intracardiac thrombosis in children with poor ventricular function, especially during rapid deterioration of ventricular function.     

Pulmonary atresia with intact ventricular septum: A quantitative cineventriculographic study of the right and left ventricular function

Summary Quantitative cineventriculographic measurements (ventricular volumes, ejection fraction, and myocardial mass) were obtained in 15 neonates with pulmonary atresia and intact ventricular septum. There was a wide dimensional range for the right ventricle, from a reduced through normal to enlarged. A restrictive tricuspid valve (less than 12 mm) was associated with a small or diminutive right ventricle. A normal function of the right ventricle was present only in those cases with normal-sized chambers. The left ventricular end-diastolic volume was always greater than normal. The ejection fraction was normal only if a normal myocardial mass was present. Additional abnormalities were frequent in the myocardium of both ventricles, such as extreme thinning of the wall of the right ventricle and hypoplasia or fibroelastosis of the left ventricle. All these factors can lead to a poor surgical prognosis despite good anatomical correction.     

单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

目的 探讨单侧肺动脉异常起源于升主动脉（AOPA）的产前超声心动图特征，提高对本病产前诊断的准确率。方法 回顾性分析2012年6月1日至2018年10月30日经首都医科大学附属北京安贞医院（我院）产前诊断的6例AOPA胎儿的超声心动图特点，总结该病胎儿期的超声心动图特征。结果 5例胎儿为右肺动脉异常起源于升主动脉（AORPA），其中3例为孤立型AORPA，2例合并Berry综合征；1例为左肺动脉异常起源于升主动脉（AOLPA），同时合并先天性肺动脉瓣缺如综合征。6例胎儿均为近端型AOPA，其中2例胎儿引产（1例AOLPA，1例合并Berry综合征）。出生的4例患儿中，1例Berry综合征患儿于出生后63 d因反复肺炎、心衰死亡；2例行AORPA根治手术，术后均恢复良好；1例刚出生，仍在随访中。近端型AOPA胎儿超声心动图特征：①多切面显示正常的肺动脉主干分叉处“八”字型结构消失,正常的肺动脉分支内径增宽；②在三血管气管切面(3VT)及左室长轴切面二维及彩色多普勒显像（CDFI）可显示升主动脉发出一粗大血管向肺内走行，该血管发出位置靠近主动脉瓣左后或右后侧壁的升主动脉；③ CDFI显示异常肺动脉分支血流孤立，与主肺动脉缺乏确切连接；④频谱多普勒显示向肺内走行的血管为肺动脉血流频谱；⑤不合并其他心内畸形时，心内结构正常。结论 超声心动图可对胎儿期的近端型AOPA做出准确的诊断，3VT及左室长轴切面对诊断近端型AOPA至关重要。     

Right Ventricular Outflow Patch Reconstruction for Repair of Double-Outlet Left Ventricle

Double-outlet left ventricle (DOLV) is conventionally repaired with an extracardiac conduit when pulmonary stenosis is present. We report a case of surgical repair for DOLV with severe pulmonary stenosis where the right ventricular outflow tract was established using a vascular graft patch anteriorly after constructing the posterior wall with autologous tissue. This approach provides excellent hemodynamic repair without the use of an extracardiac conduit and has the major advantage of growth potential.