多普勒超声心动图诊断部分性肺静脉异位引流

目的:评价多普勒超声心动图诊断部分性肺静脉异位引流(PAPVC)的价值。方法:回顾分析132例经心外科手术确诊为PAPVC患者的超声心动图诊断情况。结果:根据Brody分型,132例患者中A型14例,超声诊断11例,诊断正确率78.6%;B型103例,超声诊断57例,正确率55.3%;C型4例,超声诊断3例,诊断正确率75%;D型3例,混合型8例,术前超声均未能做出正确诊断。132例患者中右侧肺静脉异位引流90.1%;左侧肺静脉异位引流7.4%,双侧占2.5%。合并房间隔缺损(ASD)121例(91.7%),其中静脉窦型ASD 40例(33.1%),功能性单心房(大型ASD)或单心房18例(14.9%),继发孔型ASD 63例(52%);9例房间隔完整(6.8%),2例冠状静脉窦隔缺损(1.5%)。结论:彩色多普勒超声心动图可以清晰显示肺静脉的回流情况,准确诊断PAPVC。     

Factor analysis of gated cardiac blood-pool data: application to patients with congenital heart disease.

Ventricular emptying was evaluated in patients with congenital heart disease (CHD) with left-to-right (L-R) shunt by factor analysis of gated equilibrium radionuclide angiography. In 36 (95%) of 38 ventricular septal defect patients and 20 (95%) of 21 atrial septal defect patients with small L-R shunt (pulmonary to systemic blood flow, Qp/Qs less than or equal to 2.5), as well as all patent ductus arteriosus patients, two significant cardiac factors corresponding to the ventricles (ventricular factor) and the atria plus large vessels (atrial factor) were extracted. However, in all of nine ventricular septal defect patients with large L-R shunt (Qp/Qs greater than 2.5), two different ventricular factors were determined which corresponded to the right and left ventricles (RV and LV). The RV factor showed a delay of ejection phase compared with the LV factor, and the delay was correlated with the value of Qp/Qs (r = 0.82, P less than 0.01). In eight (80%) of 10 ASD patients with large L-R shunt (Qp/Qs greater than 2.5), RV was described by the two different ventricular factors located in the septal and free-wall regions. The LV was extracted in the same factor as that located in the septal region of RV. This study demonstrates the capability of factor analysis in the pathophysiological investigation of CDH with L-R shunt.     

Anomalous pulmonary venous connection of entire left lung with intact atrial septum.

Three cases of total unilateral pulmonary venous connection of the left lung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein in the superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar to what is seen in cases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the left pulmonary veins and documented the absence of an atrial septal defect.     

Brain Abscess Associated with Isolated Left Superior Vena Cava Draining into the Left Atrium in the Absence of Coronary Sinus and Atrial Septal Defect

A previously healthy 12-year-old girl presented with severe headache for 2 weeks. On physical examination, there was finger clubbing without apparent cyanosis. Neurological examination revealed only papiledema without focal neurologic signs. Cerebral magnetic resonance imaging showed the characteristic features of brain abscess in the left frontal lobe. Cardiologic workup to exclude a right-to-left shunt showed an abnormality of the systemic venous drainage: presence of isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect. This anomaly is rare, because only a few other cases have been reported.     

Right ventricular wall "ischemia" findings using a dual isotope protocol

A 74-year-old man with a history of diabetes and arterial hypertension, presented with right ventricular failure, remarkable jugular venous distension, hepatomegaly, and swelling of the lower extremities. He was complaining of atypical chest pain and was referred for an echocardiogram and a myocardial perfusion SPECT imaging study. The echocardiogram showed normal left ventricular function with a dilated right atrium and right ventricle, severe tricuspid regurgitation, pulmonary hypertension, and an atrial septal defect with bidirectional shunt. The SPECT images showed normal left ventricular function with no areas of induced ischemia but an impressive right ventricle with severe dilatation and hypertrophy. A right ventricular "perfusion abnormality," consistent with ischemic changes, seen on stress but less evident on rest images was demonstrated on the dual isotope (Tl-201 rest/Tc-99m MIBI stress) protocol but not seen on the single isotope study (rest/stressTc-99m MIBI). Coronary angiogram showed diffuse coronary atherosclerosis but without significant obstruction.     

A case of cardiac metastasis from non-Hodgkin's lymphoma demonstrating interesting findings through radionuclide studies

We have presented a quite rare case of non-Hodgkin's lymphoma developing cardiac metastasis which was demonstrated and confirmed by radionuclide studies and autopsy. A 57-year-old male with SVC syndrome underwent radionuclide angiography using 99mTc-HSA prior to 67Ga-citrate and 201Tl-chloride studies for suspected right atrial metastasis from non-Hodgkin's lymphoma. The radionuclide angiography revealed a filling defect in the right atrium. 67Ga and 201Tl studies showed increased accumulations of both nuclides in the right atrium, in congruence with the region showing a filling defect through the radionuclide angiography. These results suggest that combined radionuclide studies, such as radionuclide angiography, 67Ga and 201Tl studies, might be useful in detecting metastatic tumor in the heart.     

Scintigraphic determination of severity in pulmonary parenchymal damage in patients with atrial septal defect

According to the pattern observed on a lung perfusion scan, patients with atrial septal defect (ASD) were classified into three stages: stage I, normal perfusion (n = 15); stage II, apical prone perfusion (n = 22); and stage III, mottled perfusion (n = 23). These stages were compared with hemodynamic data obtained from cardiac catheterization and with the right ventricular ejection fraction (RVEF). Staging from I to III correlated well with pulmonary arterial pressure, and patients in stage III showed a significant increase in pulmonary arteriolar resistance and a significant decrease in Qp/Qs. There was no significant difference in RVEF between the stages. We concluded that the scintigraphic severity determined by lung perfusion scan could be valid for evaluating the extent of pulmonary parenchymal damage in patients with ASD.     

Perfusion lung scan in patients operated for transposition of the great arteries

Fifty perfusion lung scans were performed with Tc-99m albumin microspheres in 42 patients, 15 days to 12 years after corrective surgery for transposition of the great arteries. The scan was entirely normal in nine of 42 patients. Absence of left lung perfusion was observed in three patients and hypoperfusion of the left lung in 19 patients. Segmentary zones of hypoperfusion was visualized in 13 patients either in the right or left lung. A moderate right to left shunt was observed in eight cases. The absence or decrease in left lung perfusion was due to pulmonary vein occlusion in four patients, left pulmonary artery stenosis in two patients, and preferential right blood flow in one patient. Right to left shunt was due to dehiscence of the atrial patch in four patients.     

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA): A case report

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) also known as Bland-White-Garland Syndrome is a rare anomaly of coronary arteries comprising of 0.25%-0.5% of all congenital heart defects with a prevalence of 1 in every 300,000 live births. Its clinical significance lies in the possibility of resultant coronary steal phenomenon with a left-to-right shunt causing aberrant left ventricular perfusion which may ultimately lead to myocardial ischemia and infarction in children having the abnormality. ALCAPA may manifest as an isolated defect but in 5% of cases it may be associated with other cardiac anomalies such as atrial septal defect, ventricular septal defect, and aortic coarctation. We present a case of 7 years female with ALCAPA with collaterals between RCA and LCA and additional findings of juxtaposition of left atrial appendage. Juxtaposition of atrial appendage is associated with some major congenital heart diseases, transposition of great vessels being the common one. In our case, however, juxtaposition of left atrial appendage is associated with ALCAPA. Surgery is the definite treatment modality for ALCAPA available till date. Early diagnosis of ALCAPA with the help of multislice CT angiography is always good for the patient to prevent the possible grave consequences.     

Use of the Amplatzer ASD Occluder for Closing a Persistent Left Vertical Vein

We report the case of a very large anomalous connection of the veins draining the upper lobe of the left lung to both the left-sided vertical vein and the left atrium, associated with mild rheumatic mitral valve stenosis, in which the atrial septum was intact and the remaining venous system, including the coronary sinus, was otherwise normal (a variant of Lutembacher’s syndrome). In order to abolish the left-to-right shunting, a transcatheter approach to close this venous structure was successfully attempted using an Amplatzer ASD Occluder device. The technical aspects and the alternative options of performing a procedure with a device for a purpose outside the scope of its approved label are discussed.     

Scimitar syndrome: MDCT imaging revisited

Scimitar syndrome refers to a form of anomalous pulmonary venous drainage which implies partial or total failure of the pulmonary veins to reach the left atrium.AimTo emphasize the role of MDCT in the diagnosis of Scimitar syndrome.Patients and methodsWe presented MDCT imaging findings in three patients with Scimitar syndrome.ResultsMDCT revealed anomalous arterial blood supply from abdominal aorta to right lower lung lobe and anomalous venous drainage into infradiaphragmatic IVC, hypoplastic right pulmonary artery and hypoplastic right lung as well as atrial septal defect and bronchial diverticulum.ConclusionMDCT is essential investigation in Scimitar syndrome.     

Partial anomalous pulmonary venous return with secundum atrial septal defect: A case report

Partial anomalous pulmonary venous return (PAPVR) is a congenital heart anomaly in which some of the pulmonary veins return to the right atrium or one of its supplying veins instead of normally connecting with the left atrium. Oftentimes it is concurrent with a secundum atrial septal defect. PAPVR is typically asymptomatic, however symptoms of pulmonary hypertension can arise at higher degrees of left-to-right shunting. An 80-year-old male presented with exertional dyspnea and was found to have a secundum atrial septal defect on echocardiogram. A subsequent contrast enhanced computed tomography of the chest revealed a concomitant PAPVR.     

Persistent left superior vena cava to left atrial communication detected with radionuclide angiography: a case report

A persistent left superior vena cava (PLSVC) is the common clinical congenital abnormality of the greater central veins. This anomaly may occur as an isolated lesion, but rarely it is significantly associated with other congenital cardiac lesions, particularly it drain into the left atrium. We recently encountered a patient in whom PLSVC to left atrial communication with atrial septal defect was diagnosed on the basis of radionuclide angiography with Tc-99m pertechnetate. Radionuclide angiography might be the noninvasive study of choice in patients suspected of having PLSVC by the clinical findings or chest roentgenography.     

Radiological findings of partial expression pentalogy of Cantrell and other multiple congenital anomalies: A rare case report

Pentalogy of Cantrell is a rare syndrome of anomalous malformation. In the present case, the syndrome was initially diagnosed as a complete pentad, including a supra-umbilical abdominal wall defect, a sternal defect, pericardial defects, an anterior diaphragmatic defect, and heart malformation. Diagnosis required several imaging modalities, including computed tomography (CT) and magnetic resonance imaging (MRI). In this case report, we present an 8-month-old female patient with a thoracic wall defect with ectopia cordis and a bilateral cleft lip and palate. In addition, a head CT scan showed craniosynostosis, hypogenesis of the corpus callosum, and tonsillar cerebellar herniation. Thoracoabdominal CT revealed herniation of the transverse colon up to the subcutaneous layer, diaphragmatic hernia, atrial septal defects (ASD), ventral septal defects (VSD), and a persistent left superior vena cava (PLSVC). A multidisciplinary approach is required for the optimal management of this syndrome. We describe a female infant who presented with pentalogy of Cantrell syndrome and include the findings from postnatal CT imaging.     

Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.     

Multidetector CT of left atrium

MDCT angiography has enabled visualization of left atrium in addition to coronary arteries. CT images can be utilized to demonstrate normal anatomy and pathologies. Left atrium variations and pathologies include atrial septal pouch, patent foramen ovale, atrial septal defect, atrial band, lipomatous hypertrophy, cor triatriatum, diverticulum, accessory appendages, atrial thrombus and masses. Left atrium should be carefully evaluated in patients undergoing coronary CT angiography. MDCT is becoming an alternative modality for the evaluation of left atrium.     

超声心动图在房室间隔缺损经导管封堵术中监测的体会

目的探讨超声心动图在房间隔缺损经导管封堵术中监测的方法和价值。方法9例房间隔缺损(ASD)、14-例室间隔缺损(YSD)患者经胸超声心动图监测下行经心导管封堵术。结果超声监测下8例ASD封堵成功，1例因缺损口太大，封堵器无法固定而失败。14例VSD封堵成功，其中1例VSD术后有轻微残余分流。结论超声心动图在房室间隔缺损封堵术中的监测有着不可替代的重要价值。     

肺动脉闭锁伴室间隔缺损的多层螺旋CT诊断

目的:回顾性分析肺动脉闭锁伴室间隔缺损(PAVSD)的多层螺旋CT(MSCT)表现。方法:分析5例肺动脉闭锁伴室间隔缺损患者的MSCT图像。4例行平扫及双期扫描,另1例行单期扫描。结合薄层图像、多平面重组、薄层最大密度投影以及容积再现法进行观察。结果:MSCT显示肺动脉瓣闭锁2例,肺动脉干中断、无中央共汇者3例。5例均见室间隔缺损。2例见明显的支气管动脉扩张;2例见直接主动脉肺动脉侧支;2例见间接主动脉肺动脉侧支。1例合并右心室发育不良及三尖瓣狭窄,1例合并房间隔缺损;3例合并右位主动脉弓,2例合并主动脉后左无名静脉。1例有手术史者可见上腔静脉与右肺动脉吻合,与右心房无交通。结论:MSCT可清楚地显示PAVSD的主要表现。     

经导管同期封堵治疗膜周部室间隔缺损合并房间隔缺损

目的　探讨经导管介入同期治疗膜周部室间隔缺损 (VSD)合并房间隔缺损 (ASD)的可行性、方法及疗效。方法　 4例患者 ,年龄为 12～ 2 6岁。术前超声检查提示VSD合并ASD。VSD均为膜周部缺损 ,缺损直径 3～ 6mm ,缺损上缘距主动脉瓣 2～ 6mm。ASD均为继发孔型 ,缺损直径 5～ 8mm。4例患者均于术中先行左心室造影 ,确定VSD适合封堵后 ,先行VSD封堵 ,最后行ASD封堵。结果　 4例患者均经导管一次封堵治疗成功。左心室造影显示VSD呈囊袋型 3例 ,囊袋直径分别为 4、5和 10mm ,封堵器的直径分别为 4、8和 12mm。 1例缺损为管状 ,直径 3.5mm ,用 4mm的封堵器治疗成功。4例患者ASD伸展径为 6～ 10mm ,封堵器直径为 6～ 12mm。所有患者术中及术后无并发症。结论　经导管介入同期封堵治疗膜周部VSD合并ASD具有技术上的可行性、安全性和良好的治疗效果     

Quantitation of right-to-left shunt ratio in patients with pulmonary telangiectasia by technetium-99m MAA lung perfusion imaging

Two cases of pulmonary telangiectasia have been presented. Using lung perfusion scans, two quantitative estimation methods for right-to-left (R-L) shunt ratios have been described. The R-L shunt ratios estimated by lung perfusion imaging correlate well with those derived by the Fick method. The methods described are simple and accurate, and seem to be useful for evaluation and follow-up of the patients.