Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.     

Echocardiographic diagnosis of partial anomalous pulmonary venous connection from right upper lobe to the coronary sinus

A 36-year-old woman was admitted because of an enlarged right heart. Echocardiographic examination revealed an abnormal vessel connecting to the dilated coronary sinus. The abnormal vessel traveled in the direction from the right axillary to the left epigastric region. Partial anomalous pulmonary venous connection (PAPVC) from the right upper lobe to the coronary sinus was initially considered as a possible diagnosis by echocardiography. At surgery, diagnosis of an isolated PAPVC of the right upper pulmonary vein to the coronary sinus was confirmed.     

Anomalous left superior pulmonary venous connection with intact interatrial septum

Partial anomalous pulmonary venous connection (PAPVC) with intact interatrial septum is an uncommon congenital anomaly, while isolated left pulmonary venous connection with intact interatrial septum is rare. In this report, a 5-year-old girl with chief complains of mild short breath after exercise was diagnosed of anomalous connection between left superior pulmonary vein (LSPV) and left innominate vein by transthoracic echocardiography (TTE) that was confirmed by 3D cardiac CT scanning.     

Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect

Background: Partial anomalous pulmonary venous connection (PAPVC) is frequently associated with atrial septal defect (ASD), especially sinus venosus defect (SVD). Although Waggstaffe described the pathology of SVDs in 1868, the exact anatomic features and the nature of SVD remains controversial. SVDs with no posterior atrial rim were observed in recent years. However, no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD. The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD. Methods: From January 2011 to December 2019, 256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled. Comprehensive review of preoperative transthoracic echocardiography, computed-tomography images and surgical findings were performed by experienced pediatric cardiologists. The subtypes of PAPVC, locations and types of ASD, and presence of posterior atrial rim of associated ASD were investigated. Results: PAPVC was right-sided in 244 children, left-sided in 6 children, and bilateral in 6 children. In PAPVC cases, ASD without posterior atrial rim existed in 226 SVD cases. ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium (RA) or to RA-superior vena cava junction. In cases with isolated ASD, there were 3 SVD, and the other 875 cases were secundum ASD. Conclusions: ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava (SVC) junction. For SVD, the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim, rather than adjacent to the SVC or to the inferior vena cava.     

经胸和经食道超声心动图评价成人部分型肺静脉异位连接

目的通过经胸和经食道超声心动图对研究对象进行全面检查,探讨部分型肺静脉异位连接（partial anomalous pulmonary venous connection,PAPVC）的解剖学特征及其与手术的相关性。方法对2008年6月至2011年7月36例（男12例,女24例,年龄15—59岁,中位年龄37岁）经外科手术证实的先天性PAPVC患者的临床资料进行回顾性分析,所有患者均行经胸超声心动图检查。27例进一步行经食道超声心动图检查,所得诊断结果与外科手术诊断结果进行比较。结果36例PAPVC均为右肺静脉异位连接,其中两条右肺静脉21例（58．3％）,单纯右上肺静脉11例（30．5％）,单纯右下肺静脉4例（11．1％）。合并静脉窦型缺损33例（91．6％）。经胸及（或）经食道超声心动图联合检查,与手术结果对比,正确诊断PAPVC31例（86．1％）。结论超声心动图在对PAPVC的诊断中起到重要作用,为外科术前提供足够的信息,可作为PAPVC诊断的首选检查方法。     

心内型部分性肺静脉异位引流病理类型细化分型对术式选择的指导意义

目的:探讨心内型部分性肺静脉异位引流(PAPVC)病理类型的细化分型对手术方式选择的指导意义。方法:回顾分析2009年1月至2019年12月于首都医科大学附属北京儿童医院行经胸超声心动图检查诊断为心内型PAPVC并接受手术治疗的患儿46例。根据PAPVC肺静脉回流部位与房间隔缺损病理解剖特点,并结合外科治疗选择的不同术式进行细化分型。结果:(1)Ⅰ型腔静脉型33例(71.7%):回流至腔静脉-右心房结合部,此型中根据有无合并腔静脉窦型房间隔缺损再分Ⅰa骑跨型26例(56.5%),手术方式多采用房间隔修补;Ⅰb非骑跨型7例(15.2%),手术需做心内板障。(2)Ⅱ型右心房内型10例(21.7%):直接回流至右心房后壁,手术也需做心内板障。(3)Ⅲ型冠状静脉窦型3例(6.5%):回流至冠状静脉窦,手术需切开冠状静脉窦壁。结论:PAPVC细化后的病理亚型可以指导手术方式的精确选择。     

Massive pulmonary embolism immediately diagnosed by transthoracic echocardiography and treated with tenecteplase fibrinolysis

We report the case of a 75-year-old woman with new onset dyspnoea, hypotension, and right bundle branch block. Transthoracic echocardiography (TTE) showed a thrombus in the right pulmonary artery and acute pulmonary embolism was diagnosed. The patient immediately underwent fibrinolysis with tenecteplase, with prompt recovery of clinical conditions and ECG anomalies. Bedside TTE might be helpful for immediate diagnosis of massive PE needing rapid treatment by fibrinolysis.     

Mitral Stenosis, Sinus Venosus Atrial Septal Defect, and Partial Anomalous Pulmonary Venous Return

We describe a 42-year-old man with rheumatic mitral stenosis, sinus venosus atrial septal defect, and anomalous drainage of the right upper pulmonary vein to the superior vena cava. Transthoracic echocardiography (TTE) failed to identify the atrial septal defect and the partial anomalous pulmonary venous return. Transesophageal echocardiography (TEE), using a multiplane probe, was useful in delineating the abnormalities. To our knowledge, this is the first reported patient with rheumatic mitral stenosis and sinus venosus defect.     

Is it Safe to Close ASD with the Guidance of Transthoraxic Echocardiography in Pediatric Population “Ten Years' Experience of a Single Center”

Objective

Main purpose of this study is to emphasize the usage and safety of transthoracic echocardiography (TTE) in percutaneous atrial septal defect (ASD) closure in appropriate pediatric cases.

Background

Nowadays, percutaneous closure is preferred as treatment modality for ASD in pediatric age group.

Methods

Between the dates December 2003–August 2013; 340 patients whose ASD were closed included in this study. Physical examination, electrocardiogram, TTE were done before the procedure, at the 24th hour, 1st and 6th month after the procedure. After the 6th month, routine control was done annually. Transesophageal echocardiography (TEE) was performed in 184 cases with large, multifenestrated ASD and when TTE views were poor in quality. We selected the 201 patients whose ASD diameter between 10–20 mm and formed 2 homogeneous groups according to the type of echocardiography used (TEE or TTE) in order to compare the role of echocardiography.

Results

The demographic features of patients of 2 groups were similar. There was not any statistically difference between ASD, balloon sizing diameters between the groups. No statistically significant difference in the success, complication, and residual shunt rates was found between the groups. Procedure, fluoroscopy time, and amount were significantly higher in TEE group. When hemodynamic variables except pulmonary blood flow to systemic blood flow (Qp/Qs; right–left atrium mean pressure, pulmonary vascular resistance, and peak‐mean pulmonary arterial pressure) were compared, there was not statistically difference.

Conclusion

TEE is an invasive procedure and requires general anesthesia, therefore, it should not be done routinely in ASD but only in selected cases. If the size and the anatomy of ASD is appropriate, TTE should be preferred primarily in percutaneous ASD closure.

     

Comparison of Intracardiac and Extracardiac Malformations Associated with Single Atrium,Single Ventricle and Single Atrium-Single Ventricle Using DualSource Computed Tomography

Background: To evaluate the qualitative and quantitative differences between intracardiac and extracardiac vascular malformations in patients with a single atrium (SA), single ventricle (SV) and single atrium-single ventricle (SA-SV) using dual-source CT (DSCT), and to compare the diagnostic performances of DSCT and transthoracic echocardiography (TTE). Methods: This retrospective study included 24 SA, 75 SV and 24 SA-SV patients who underwent both DSCT and TTE before surgery. The diagnostic values of DSCT and TTE for intracardiac and extracardiac malformations were compared according to the surgical results. The diameters of the major artery and vein were measured and calculated based on DSCT and compared among the three groups. Results: The most common malformation was pulmonary artery disease in SA (50.0%) and SA-SV (45.8%) groups and patent ductus arteriosus (33.3%) in SV group. Although there was no statistical difference, arterial development was relatively poor in the SA group. All groups showed the trend of pulmonary artery stenosis (SAvs. SVvs. SA-SV: 50.0% vs. 30.7% vs. 33.3%). There was a significant difference in mean pulmonary vein index among the groups (p = 0.017). The diagnostic sensitivity of DSCT was superior to that of TTE for extracardiac malformations. Conclusions: The most common malformation in SA and SA-SV patients is pulmonary artery stenosis. SV patients are most likely to be complicated with patent ductus arteriosus. DSCT is more advantageous than TTE for diagnosing combined extracardiac malformations and can accurately measure the diameter of arteriovenous vessels.     

Role of transthoracic Doppler pulmonary venous flow pattern in large atrial septal defects

OBJECTIVE: Large uncomplicated atrial septal defect (ASD) alters the pulmonary venous flow (PVF) pattern. We aimed to study the role of transthoracic echocardiography (TTE) in estimating the PVF Doppler abnormalities in ASD. By repeating the study soon after ASD closure, we correlated the hemodynamics of atrial shunting with PVF patterns. METHODS: This study was performed in a tertiary care referral teaching hospital in 2003. The TTE PVF patterns of 34 patients with ASD were studied. TTE study was reported by two blinded investigators independently. Surgical closure of ASD was done on eleven patients and the early postoperative PVF Doppler pattern was also studied with TTE. RESULTS: PVF patterns were adequately recorded in 34 of 38 (90%) subjects with ASD with equal male: female ratio (n = 17 each). The mean age of the study group was 21.4 +/- 8.7 years. ASD ranged from 10 to 38 mm in diameter with a mean of 18 +/- 4.2 mm. Continuous antegrade wave (CAW, mean 68.45 +/- 13.6 cm/s) replaced normally occurring S and D waves in all ASD patients. The atrial reversal wave was reduced or absent (mean 20.18 +/- 3.28 cm/sec). After ASD closure, the CAW was replaced by the S (46.18 +/- 7.5 cm/sec) and D waves (57.72 +/- 9.7 cm/sec) with increase in atrial reversal wave to 27.81 +/- 5.1 cm/sec. CONCLUSIONS: The S and D antegrade waves normally seen in PVF are replaced by a continuous antegrade wave in ASD. Atrial reversal wave is also reduced. PVF waveform becomes normal after ASD closure. TTE PVF Doppler pattern can help estimate ASD hemodynamics.     

Coexistence of Primary Sarcomatoid Carcinoma of the Right Ventricle and Absence of Right Pulmonary Artery

We describe the imaging features of a 48‐year‐old woman with primary sarcomatoid carcinoma originating from right ventricular outflow tract (RVOT) and isolated absence of right pulmonary artery (RPA). Computed tomography pulmonary angiography (CTPA) demonstrated the absence of RPA. Both transthoracic echocardiography (TTE) and CTPA revealed multiple masses filling and obstructing the RVOT. A palliative operation was performed on the patient, and the postoperative histopathology and immunohistochemical examination confirmed primary sarcomatoid carcinoma originating from the RVOT. The operation also confirmed the absence of RPA.     

The role of transesophageal echocardiography in the management of patients with acute and chronic pulmonary thromboembolism

Acute and chronic pulmonary thromboembolism carry high mortality. The role of transesophageal echocardiography (TEE), however, has not been well delineated in patients with suspected pulmonary thromboembolism. The aim of the present study was to demonstrate the value of Tee in patients with various clinical manifestations of pulmonary thromboembolic disease. Twelve patients--ten males and two females, age 47-85 years--are presented in whom central pulmonary thromboembolism was found by TEE. Six patients were referred for breathlessness and had moderate to severe pulmonary hypertension (PH) with (3) or without (1) right atrial thrombus or had right heart dilatation (1) or right ventricular myxoma (1) on transthoracic echocardiography (TTE). Thrombolysis (2), surgery (2), and heparin (2) treatment was performed without angiography. All but one patient recovered. Six patients had severe PH by TTE, one of them had a right atrial thrombus. Angiography was done in five patients in whom surgery was considered. Pulmonary thromboendarterectomy was successfully performed in two patients, it was contraindicated in two patients for advanced age or severe left ventricular dysfunction, both patients died during follow-up, and two patients were waiting for surgery. In conclusion: TEE has a definite role in the management of patients with acute pulmonary thromboembolism or in pulmonary embolism associated with right-sided intracardiac masses and in the selection of patients with PH for pulmonary thromboendarterectomy.     

Three-dimensional echocardiography assessment of carcinoid valvular heart disease: Images of each and all

A 54-year-old male was found to have neuroendocrine carcinoma with hepatic metastasis. Two-dimensional (2D) transthoracic echocardiography (TTE) demonstrated dilated right ventricle and right atrium, and severe tricuspid and pulmonary regurgitation. Three-dimensional (3D) TTE en-face views showed thickened, retracted, and fixed tricuspid valve and pulmonic valve which remained widely open throughout the cardiac cycle. 3D TTE, particularly en-face views, demonstrates incremental value over 2D TTE by providing precise valvular anatomic details comparable to surgical findings. 3D TTE also offers a unique opportunity to assess all four valves simultaneously with en-face views to delineate their relationships with surrounding structures.     

Percutaneous Occlusion of Right Partial Anomalous Pulmonary Venous Connection with Dual Drainage to the Innominate Vein and the Left Atrium: A Unique Anatomical Finding

A 43-year-old woman with a past medical history of aortic coarctation surgically repaired at the age of 3 years using an end-to-end anastomosis, presented with 2 years complain of increasing dyspnea and fatigue with exercise associated to frequent palpitations. During extensive work-up, she was found to have a partial anomalous pulmonary venous connection (PAPVC) with “dual drainage” represented by a communication between the right pulmonary veins draining into the left atrium and the innominate vein via an anomalous vein due to a persistence of early connections between the sinus of the right pulmonary veins and the cardinal veins system in the splanchnic plexus and also a persistence of the proximal portion of the left anterior cardinal vein. She was successfully treated through a percutaneous implantation of a vascular plug occluding the vertical portion of the anomalous vein diverting the flow to the left atrium. To the best of our knowledge, this anatomical variant of partial anomalous pulmonary venous connection with dual drainage has not been previously reported.     

Pedunculated Pulmonary Artery Sarcoma Suggested by Transthoracic Echocardiography

Pulmonary artery sarcoma (PAS) is an extremely rare malignancy. It is usually found after it grows large enough to occupy almost the entire lumen of the pulmonary artery and causes serious clinical symptoms. Thus, it is usually difficult to distinguish PAS from pulmonary thromboembolism based on imaging examinations. Few case reports had shown the attachment of PAS to pulmonary artery, a key characteristic for diagnosis, and differential diagnosis of PAS. In this case, we found a PAS, which did not cause local obstruction and some tumor emboli, which obstructed the branches of the pulmonary arteries and caused pulmonary hypertension and clinical symptoms. Transthoracic echocardiography (TTE) revealed a part of the tumor attached to the intima of the main pulmonary artery with a peduncle and had obvious mobility, which was suggestive of PAS and differentiated it from the pulmonary thromboembolism. To our knowledge, this is the first case report of a pedunculated PAS suggested by TTE. Combined with pulmonary artery computed tomography angiography, the diagnosis of PAS is strongly suggested before the operation. This case indicates that TTE could reveal the attachment and mobility of PAS in the main pulmonary and may provide useful information for the diagnosis and differential diagnosis of PAS, especially a pedunculated PAS.     

Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

We report the management of a patient with secundum atrial septal defect (ASD) and severe pulmonary hypertension. A 65-year-old male with recently diagnosed atrial septal defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension. Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7. An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure. The implantation of a home-made fenestrated Amplatzer ASD Occluder (ASO) was planned in order to decrease left-to-right shunt and promote further decrease of pulmonary arterial pressure in the long-term. Thus, by means of mechanical intracardiac echocardiography study with a 9F 9 MHz UltraIce catheter (Boston Scientific Corp.), we selected a 34 mm ASO for implantation. Four millimeter fenestration was made inflating a 4 mm non-compliant coronary balloon throughout the waist of the ASO, which was successfully implanted under intracardiac echocardiography. After six months, a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination. This case suggests that transcatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.     

Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Background and objective: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. Methods: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, USA between October 2005–2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. Results: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. Group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. Conclusions: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.     

Transthoracic Echocardiography for Diagnosis of Right Pulmonary Artery to Left Atrial Fistula

Right pulmonary artery to left atrial fistula (RPA‐LAF) is a rare cardiovascular anomaly. There were no systematic and detail represent by echocardiography. We chose the patients who diagnosed with RPA‐LAF at Fuwai Hospital from 2000 to 2010. All patients underwent clinical exanimation, chest roentgenogram, laboratory testing, electrocardiography, transthoracic echocardiography (TTE), contrast echocardiography, and cardiac catheterization. In this article, we summarize the characteristics of the TTE for diagnosing the rare cardiovascular anomaly of RPA‐LAF. We undertook a detailed review of their TTE and contrast echocardiography findings to determine the characteristic findings of this condition.     

“Gigantic” biatrial myxoma with right heart functional impairment

Cardiac myxomas are frequently located in the left or right atria, with multiple locations being rare. We report a 59‐year‐old healthy female with 5 months of cough and exertional dyspnea. A transthoracic echocardiography (TTE) exhibits a 9 × 5 cm nonpedunculated tumor arising from the interatrial septum (IAS) and inhabiting both atria, but was unable to depict the relation with the IAS. Transesophageal echocardiography exposes a single tumor crossing the IAS through an ostium secundum atrial septal defect (ASD) causing right heart functional impairment. Uneventful cardiac surgery allowed complete resection of the lesion and ASD closure. Pathology reported a myxoma.