产前超声诊断胎儿右房异构一例

本文报道产前超声诊断胎儿右房异构一例。孕妇孕24周产前超声检查发现胎儿左位心合并复杂心血管畸形(右心室双出口、房室间隔缺损、肺动脉发育不良、双侧上腔静脉、心下型完全型肺静脉异位引流)、胃泡位于腹腔右侧、中位肝、可疑无脾、腹主动脉与下腔静脉位于脊柱左侧、双侧支气管呈右侧支气管对称形态,综合考虑右房异构可能。引产后经尸体解剖证实脾脏发育不良、右房异构。右房异构常合并复杂心血管畸形,因此产前超声发现复杂心血管畸形时,应警惕右房异构的可能。右房异构病死率极高,产前诊断具有重要意义。     

产前超声诊断在胎儿左侧异构中的应用

目的:探讨胎儿左侧异构的产前超声征象及其诊断价值。方法:回顾分析21例产前诊断为胎儿左侧异构的病例资料,对比总结其超声征象与随访结果。结果:21例左侧异构胎儿中,内脏心脏异位16例,先天性心脏病19例(伴心脏传导阻滞5例),下腔静脉离断伴奇静脉异常连接19例,其中两种及两种以上异常诊断胎儿左侧异构的敏感性为90.5%。染色体异常3例,18三体2例,13三体1例。18例终止妊娠,1例胎死宫内,2例存活。结论:产前超声诊断胎儿左侧异构是可行的,重要超声征象是内脏心脏异位、先天性心脏病伴或不伴心脏传导阻滞、下腔静脉离断伴奇静脉异常连接,出现上述两种或两种以上异常可提高该病的诊断敏感性。     

Cardiac and other malformations in parapagus twins

Introduction Conjoined twins occur in one in 50–200,000 live births and approximately 75% of all cases are thoracopagus twins. Parapagus conjoined twins are rare and are not included in many review articles.Case report The cardiovascular system of a male parapagus (dicephalus, tetrabrachius, dipus) conjoined twins was studied. Twin B had bilateral bilobed lungs and a normal heart, while bilateral trilobed lungs and right isomerism was found in twin A. They shared a diaphragm and an abdominal cavity. There were two complete heads on two necks, two thoraxes, one abdomen, four arms, two legs, two complete vertebral columns, a single pelvis, and severe cardiac anomalies.     

The effect of a systemic arteriovenous fistula on the pulmonary arterial blood pressure in the fetal sheep.

In order to investigate whether systemic arteriovenous fistula occurring during the fetal period could induce pulmonary hypertension at birth, a fistula was surgically created between the carotid artery and jugular vein of fetal lambs at 120 days' gestation. Mean pressures in the left pulmonary artery, aorta, atrium and ventricles were measured at birth in seven experimental animals and in five control animals. Mean left pulmonary pressure was significantly higher in the lambs with fistula as compared with the control group, suggesting that prenatal occurrence of systemic arteriovenous fistula may induce fetal pulmonary hypertension. The present study provides a new animal model that could be relevant for the study of mechanisms regulating pulmonary vascular tone in the perinatal period.     

Doppler study on pulmonary venous flow in the human fetus

OBJECTIVES: The fetal pulmonary circulation is different from the postnatal circulation: the flow is small. The arterial pressure is almost systemic, and the vascular resistance is high. Moreover, lungs are collapsed in the fetus. However, the pulmonary venous flow in the fetus has not been studied. The aims of this study were to look at flow patterns of pulmonary venous system in the fetus and to see whether there are any changes in Doppler parameters with fetal growth. METHODS: The pulmonary venous flow velocity was analyzed in 56 normal human fetuses. The fetal gestational age ranged from 17 to 37 weeks. Pulsed-Doppler echocardiography was used to investigate the right upper pulmonary vein in four-chamber view. After the pulmonary venous signal was obtained, by enlarging the sample size, the pulmonary arterial signal was superimposed for timing. Heart rate velocities at systolic peak (S), at diastolic peak (D), at nadir between S and D (O), and at nadir between D and the next S (X) and velocity-time integral were measured. RESULTS: The pulmonary venous signal showed basically continuous flow from pulmonary vein toward left atrium through all the cardiac cycles, and it had biphasic peaks: one during the systolic and the other during the diastolic phase. The systolic peak of pulmonary vein occurred slightly earlier or later than the systolic upstroke of pulmonary arterial waveforms. The systolic peak of pulmonary vein was higher than the diastolic peak in 45 out of 56 cases. Velocities of systolic and diastolic peaks and velocity-time integral of the pulmonary vein have significantly increased with fetal growth. CONCLUSIONS: In the fetus, the flow pattern of the pulmonary vein was similar to that of the postnatal pattern with the exception of absence of atrial reversal. Lower velocities may reflect a decreased pulmonary flow volume.     

Intracardiac resection of a metastatic choriocarcinoma.

BACKGROUND: Postpartal choriocarcinoma is a rare complication of pregnancy, which usually responds well to chemotherapy, even in advanced stages. Metastatic heart involvement typically characterizes a widely advanced disease, requiring palliative treatment. CASE: A 41-year-old woman with disseminated choriocarcinoma following a full-term pregnancy presented with clinical signs of severe dyspnea. Pulmonary hypertension and a floating left atrial tumor were diagnosed upon echocardiography. Tumor resection was performed by intracardiac surgery. Histological examination revealed a metastatic thrombus of a choriocarcinoma that had obstructed the right inferior pulmonary vein at the entrance to the left atrium. The patient was treated with high-dose chemotherapy. After a 1-year follow-up the patient continues to be in full remission. CONCLUSION: Tumor reductive surgery followed by high-dose chemotherapy might be a useful approach in patients with metastatic choriocarcinoma even in cases of rare tumor sites.     

Acquired heart block: a possible complication of patent ductus arteriosus in a preterm infant

A large patent ductus arteriosus (PDA) is a frequently encountered clinical problem in extremely low birth weight (ELBW) infants. It leads to an increased pulmonary blood flow and in a decreased or reversed diastolic flow in the systemic circulation, resulting in complications. Here we report a possible complication of PDA not previously published. On day 8 of life, a male ELBW infant (birth weight 650 g) born at a gestational age of 23 weeks and 3 days developed an atrioventricular block (AV block). The heart rate dropped from 168/min to 90/min, and the ECG showed a Wenckebach second-degree AV block and intraventricular conduction disturbances. Echocardiography demonstrated a PDA with a large left-to-right shunt and large left atrium and left ventricle with high contractility. Within several minutes after surgical closure of the PDA, the heart rate increased, and after 30 min the AV block had improved to a 1:1 conduction ratio. Echocardiography after 2 h revealed a significant decrease of the left ventricular and atrial dimensions. Within 12 h, the AV block completely reversed together with the intraventricular conduction disturbances. We suggest that PDA with a large left-to-right shunt and left ventricular volume overload may lead to an AV block in an ELBW infant. Surgical closure of the PDA may be indicated.     

Management of intracavitary left atrium tumors during pregnancy - two case reports.

OBJECTIVE: Authors documented an individual management of intracavitary left atrium tumors diagnosed during pregnancy. SUBJECT: Two case reports were presented. Brain embolisation was supposed in the case one of intracavitary left atrium tumor. An urgent cardiosurgery at 24 weeks' gestation was performed on the cardiopulmonary bypass. In case two (multiple pregnancy - twins) cardiac tumor in left atrium was detected in third trimester of pregnancy. The mother was without any serious cardiac and systemic complications during the last trimester. Surgical approach was different - removal of tumor after delivery. CONCLUSION: The surgical approach should be determined by clinical behavior of left atrial cardiac tumors.     

Endotoxin shock in the beagle dog.

The hemodynamic and metabolic responses to endotoxemia were assessed in seven splenectomized, chronically instrumented, conscious but lightly sedated beagle dogs. Measurements included: cardiac output (CO), right and left atrial pressures (RAP, LAP), systemic and pulmonary arterial pressures (SAP, PAP), and heart rate (HR). Arterial blood samples were measured for blood gas tensions, pH, lactate, and pyruvate. After a brief control period, 1 mg/kg endotoxin (Difco 026:B6) was given as an intravenous bolus. Two hours later, each animal was treated with low-molecular weight dextran (LMDX), 2 ml/kg/min for 15 minutes. Endotoxin initially produced a precipitate decline in CO, HR, RAP, LAP, and SAP with concurrent pulmonary hypertension; both pulmonary and systemic vascular resistance increased significantly, then declined to control values as the animal partially recovered. A progressive metabolic acidosis with excess lactate accumulation developed. LMDX produced a significant increase in CO, SAP, PAP, and LAP with a decrease in HR; RAP increased slightly. With hydration, hemodilution was noted along with relief of the metabolic acidosis and the oxygen debt. We conclude (1) neither pulmonary nor systemic vasconstriction persisted in the shocked dog, (2) the response of the pulmonary and systemic vascular beds to endotoxin was qualitatively similar, and (3) oncotic fluid therapy appeared to restore the hemodynamic and metabolic parameters to preshock values.     

Heterogeneity in fetal akinesia deformation sequence (FADS): autopsy confirmation in three 20-21-week fetuses.

Fetal akinesia deformation sequence (FADS) is a rare condition characterized by intrauterine growth retardation (IUGR), congenital limb contractures, pulmonary hypoplasia, hydramnios and craniofacial abnormalities. The present report comprises an autopsy study of three fetuses to illustrate the variable clinical manifestations and neuropathological findings. Fetus 1 had arthrogryposis and no movement on fetal ultrasound examination. Aborted at 21 weeks, the fetus showed micrognathia, bilateral joint contracture with pterygia at the elbow and axilla. Growth retardation and pulmonary hypoplasia were not major features. Neuropathologic examination revealed anterior horn cell loss and lateral corticospinal tract degeneration in spinal cord, with marked muscular atrophy. Fetus 2, 20 weeks' gestation, had fetal akinesia, nuchal thickening, left pleural effusion, and Dandy-Walker malformation on ultrasound examination. Autopsy showed low-set ears, ocular hypertelorism, cleft palate, flexion contractures with pterygia over axilla, elbow and groin, pulmonary hypoplasia, Dandy-Walker malformation, unremarkable spinal cord and skeletal muscle. Fetus 3, 21 weeks' gestation, was aborted for fetal akinesia, neck and limb webbing and severe arthrogryposis. At autopsy, similar facial abnormalities, contracture and pterygia in neck and multiple major joints were found. Borderline pulmonary hypoplasia and severe lumbar scoliosis were also present. The brain, spinal cord and muscle were unremarkable. In these three fetuses, the prenatal ultrasound and autopsy findings were characteristic of FADS. Neurogenic spinal muscular atrophy was the basis of fetal akinesia in Case 1. Dandy-Walker malformation was present in Case 2, but the pathogenetic mechanism of fetal akinesia was not clear as spinal cord and muscle histology appeared normal. The etiology of akinesia was undetermined in Case 3; no extrinsic or intrinsic cause was identified.     

Fetal complete heart block: antenatal diagnosis, significance and management

Complete heart block was diagnosed prenatally in 21 fetuses. Associated structural cardiac defects were present in 18 fetuses, in particular complete atrioventricular canal with atrial isomerism (5 cases), and 'corrected' transposition of the great arteries (4 cases). Maternal systemic lupus erythematosus was proved in only one case. In 11 fetuses, intra-uterine congestive heart failure with the signs of non-immune hydrops fetalis occurred. In all 11 fetuses, the hydrops was associated with a cardiac defect, in particular complete atrioventricular canal with atrial isomerism in 5 cases. A review of the literature confirms that only the association of complete heart block and cardiac malformation can cause intra-uterine congestive heart failure, whereas in the case of fetal complete heart block without cardiac malformation or with prenatally hemodynamically insignificant cardiac malformation, congestive heart failure is rare. Only 30% of newborns with complete heart block have associated cardiac malformations. In our series, however, 86% of the fetuses with complete heart block had cardiac malformations. The most important reason for this percentage discrepancy is that almost all fetuses with associated severe cardiac defects, in particular atrioventricular canal defects, develop heart failure which frequently results in prenatal death. Thus, fetal deaths are not included in pediatric statistics. Nevertheless, fetuses with isolated complete heart block generally do not develop heart failure and in almost all of the cases are born alive.     

Treatment with new surgical method for chronic atrial fibrillation in 5 cases with mitral valve disease]

Chronic atrial fibrillation is the most common arrhythmia in patients with mitral valve disease, it not only deteriorates cardiac output, but also promotes a 5.6 times higher incidence of thromboembolic events. Nevertheless, the exact mechanism of atrial fibrillation is still poorly understood. Moreover, the long-term results of treatment, either by drugs or cardioversion, are almost always disappointing. Recent investigations suggest that atrial fibrillation is probably due to intraatrial multiple reentrant waves. It is also well-known from animal experiments that for sustenance of the fibrillation a critical mass of atrium is necessary. Based on these data, a new surgical method has been carefully designed to reduce atrial mass and to prevent sustenance of fibrillation. Between July and December 1988, five patients, one male and four females, with mitral valve disease and chronic atrial fibrillation were selected for this new surgical method. The age distribution ranged from 25 to 57 years, with a mean of 36 +/- 13 years. All of them had chronic atrial fibrillation which had been documented for more than one year. The operation was performed using the cardiopulmonary bypass and cardioplegia myocardial protection techniques. The left atrium was opened via the interatrial sulcus. After the mitral valve was repaired, cryosurgery (-60 degrees C for 120 sec) was applied along the borders of the left atrial free-wall. The left atrium was separated by these cryolesions into two sections, each about half the mass of the original and both sections were connected with a few atrial fibers located behind the coronary sinus. No surgical mortality or complications were encountered.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fetal cardiac interventions

Fetal cardiac interventions are being performed with growing success by a minimally invasive percutaneous and transthoracic approach. The primary aim of these interventions is to minimise postnatal morbidity and mortality, rarely also to achieve intrauterine survival. Valvuloplasty in utero for severe aortic stenosis is performed in order to achieve sufficient growth of the left ventricle and to make a later biventricular repair possible. In rare cases with hydrops secondary to massive left ventricular dilatation and mitral insufficiency it is used as a salvage therapy. Premature obstruction of the foramen ovale can be treated by balloon atrioseptoplasty or stenting of the atrial septum with the aim to attain a decompression of the left atrium and consequently of the pulmonary veins. This might reduce the extent of pulmonary hypertension and the resulting vascular and parenchymal changes in affected infants. Intrauterine valvuloplasty of a highly stenotic pulmonary valve or a pulmonary atresia with intact ventricular septum in order to prevent hypoplasia of the right ventricle and to enable postnatal biventricular repair is only rarely justified. Currently these intrauterine cardiac interventions are limited to a small group of fetuses with cardiac defects. However, with enhanced imaging modalities and equipment and with growing experience, fetal cardiac interventions are likely to increase in the next years.     

Results of Senning operation for transposition of great arteries.

We report our results with 10 infants and children who underwent atrial repair using the Senning operation between 1985 and 1990. All cases had abdominal situs solitus, levocardia and atrio-ventricular concordance (D-bulboventricular loop). Nine patients had simple D-transposition of the great arteries without ventricular septal defects (VSD) or left ventricular outflow tract obstruction. The other patient had a double outlet right ventricle with subpulmonic VSD and pulmonary hypertension (PHT) and underwent a palliative Senning procedure. All patients had a balloon atrial septostomy (BAS) before surgery, except for one with Taussig-Bing syndrome. One patient had a Blalock-Hanlon operation after BAS. The age at the time of surgery ranged from two months to four years seven months (mean: 22 months) and weight ranged from 4.3-12 kg. There were two hospital mortalities including the patient with VSD and PHT. All of the patients had echocardiographic examinations and six of the eight survivors received cardiac recatheterization four to 19 months (mean: 7.4 months) postoperatively. No baffle leaks were noted in the survivors. Two patients had both mild tricuspid regurgitation and slightly decreased right ventricular contractility, and one patient had a pressure gradient of 6 mmHg between the superior vena cava and neo-right atrium. The clinical follow-up interval was eight to 64 months (mean: 31.4 months). All survivors showed a sinus rhythm on their latest electrocardiogram and were participating in normal daily activities without medication.     

Reference values for the volumes of foetal heart atrial wall by three-dimensional ultrasound using STIC and VOCAL methods between 20w0d and 33w6d weeks of gestation

Objective: To establish reference values for the volumes of foetal heart atrial wall by three-dimensional (3D) ultrasound using spatio-temporal image correlation (STIC) and virtual organ computer-aided analysis (VOCAL) methods.

Methods: We performed a retrospective cross-sectional study with 170 normal singleton pregnancies between 20 weeks?+?0 days (20w0d) and 33 weeks?+?6 days (33w6d) of gestation. Foetal heart atrial wall volume was obtained by VOCAL method with 30-degree rotation (six planes) subtracting the internal volume from the atrium volume. Polynomial regression with adjustments by determination coefficient (R²) was performed. To calculate the interobserver reproducibility, concordance correlation coefficient (CCC) was applied.

Results: The mean?±?standard deviation (SD) for the left atrium wall volume (cm³) ranged from 0.54?±?0.21 at 20w0d–20w6d to 2.17?±?0.30 at 33w0d–33w6d. The mean?±?SD for the right atrium wall volume (cm³) ranged from 0.45?±?0.16 at 20w0d–20w6d to 2.17?±?0.62 at 33w0d–33w6d. We observed a satisfactory interobserver reproducibility with CCC?=?0.69 and 0.58 for the left and right volumes of foetal heart atrial wall, respectively. The best-fit models were first-degree: volume for the left atrium wall?=??2.194?+?0.139*GA (R^2?=^?0.41) and volume for the right atrium wall?=??2.757?+?0.155*GA (R^2?=^?0.37).

Conclusion: Reference values for the volumes of foetal heart atrial wall by 3D ultrasound using STIC and VOCAL methods between 20w0d and 33w6d weeks of gestation were established.     

先天性心脏病患儿血清儿茶酚胺 醛固酮测定临床意义研究

摘要：目的　研究小儿左向右分流型先天性心脏病（CHD）血清儿茶酚胺（CA）［肾上腺素（E）、去甲肾上腺素（NE）、多巴胺（DA）］和醛固酮（ALD）的变化,分析其与心功能、容量负荷、肺动脉压力及心肌重塑的相关性。 方法　2007年10月至2008年10月天津市儿童医院心脏科住院经彩色多普勒超声心动图检查确诊为左向右分流性CHD患儿53例,对照组20例,检测血清CA、ALD。采用实时三维超声心动图检测心脏房室腔径及心功能指标。 结果　（1）CHD组血清NE、ALD高于对照组（P < 0.01）。（2）心衰组、大分流组、肺动脉高压组血清CA、ALD高于无心衰组、小分流组、无肺动脉高压组（P < 0.01）。（3）心衰组患儿左室舒张末期内径指数、左室舒张末期容积指数、左室质量指数均高于无心衰组（P < 0.01）。（4）血清CA与左房内径指数、左室舒张末期内径指数、左室舒张末期容积指数呈正相关（P < 0.01）。 结论　血清CA、ALD可作为早期诊断心力衰竭及肺动脉高压的血清学指标之一,CA与心肌重塑密切相关。     

Early discontinuation of intravenous prostaglandin E1 after balloon atrial septostomy is associated with an increased risk of rebound hypoxemia.

OBJECTIVE: A comparison of the effects of early (<2 h) and late (>or=2 h) discontinuation of prostaglandin E1 (PGE1), on systemic oxygenation following a successful balloon atrial septostomy (BAS), in neonates with confirmed diagnosis of d-transposition of the great arteries (d-TGA). STUDY DESIGN: Neonates with a postnatal diagnosis of d-TGA who were admitted to a quaternary neonatal intensive care unit between January 1999 and December 2004 were identified from the local database. The effects of time of discontinuation of PGE1 on oxygen saturations, oxygen requirement, need for reinstitution of prostaglandin infusion and postoperative stability were analyzed. RESULT: Sixty neonates with a diagnosis of d-TGA were identified, 45 of whom had a BAS performed. Of these, 25 cases had early (<2 h) discontinuation of PGE1 whereas in the remaining 20 discontinuation was late (>or=2 h). PGE1 infusion was recommenced in 20 neonates (20/45 (44%)) after a successful BAS due to rebound hypoxemia. Of these, there was a threefold increase in the need for reinstitution of prostaglandin in the early compared to late discontinuation group (16/25 (64%) vs 4/20 (20%), P<0.006). There was no difference in postoperative cardiorespiratory stability. CONCLUSION: Early discontinuation of intravenous PGE1 following BAS was associated with an increased risk of rebound hypoxemia, necessitating the recommencement of PGE1. We speculate the rapid improvement in oxygenation on reinstitution of PGE1 is secondary to pulmonary vasodilation and improved pulmonary blood flow. We propose a more cautious and graded approach to discontinuation of PGE1 based on illness severity and the magnitude and duration of hypoxemia at presentation.     

Simultaneous tubal pregnancy and twisted ovarian cyst

Background Simultaneous or concomitant bilateral adnexal pathology is rare. But simultaneous tubal pregnancy and twisted ovarian cyst is even rarer.Case A 25-year-old woman, gravida 2, parity 0, presented with acute abdomen after 12 weeks of amenorrhea. Simultaneous right tubal pregnancy and twisted left ovarian cyst were intraoperatively diagnosed. Right salpingostomy and left salpingo-oophorectomy were performed. The follow-up serum beta-hCG was negative at the nineteenth postoperative day. She was well at discharge and throughout the 4-week follow-up period.Conclusion Although simultaneous or concomitant bilateral adnexal pathology is uncommon, a careful assessment of both adnexa is mandatory especially in the cases with inconsistent site of symptom and pathology in order to avoid undiagnosed simultaneous pathologies that may be missed.     

Coexistence of tuberculous constrictive pericarditis and right atrial tuberculoma: a case report.

Tuberculous constrictive pericarditis is a rare condition with a high mortality rate. The coexistence of constrictive pericarditis and intracardiac tuberculoma has not previously been reported. We report the case of a 65-year-old man presenting with left-side pleural effusion and signs of systemic venous congestion for 2 months. Echocardiography and computerized tomography showed a thickened pericardium and a mass in the right atrium. Pericardiectomy and excision of the right atrial mass were performed. Pathologic examination of the pericardium and the right atrial mass both revealed chronic granulomatous inflammation with acid-fast bacilli and confirmed the diagnosis of tuberculous constrictive pericarditis and right atrial tuberculoma. This case reminds us of the possibility of this type of rare combination of tuberculous constrictive pericarditis and intracardiac right atrial tuberculoma, and the need for complete imaging studies when such cases are encountered.