Transthoracic Echocardiographic Assessment of Spindle Cell Sarcoma of the Pulmonary Artery in a Child

In this report, we present a case of spindle cell sarcoma of the pulmonary artery diagnosed by transthoracic echocardiography. To the best of our knowledge, this case is the youngest reported case of pulmonary artery sarcoma (PAS) to date. PAS is frequently confused for pulmonary embolism; in this case, echocardiographic findings allowed for differentiation between pulmonary embolism and solid tumor.     

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.     

Transesophageal Three-Dimensional Color Doppler Echocardiographic Reconstruction of the Left Vertebral Artery

We present a patient in whom we successfully reconstructed a long segment of the proximal left vertebral artery in three dimensions using color Doppler transesophageal echocardiography (TEE). Three-dimensional (3-D) color Doppler TEE may complement two-dimensional (2-D) TEE by its ability to view cross sections of the left vertebral artery at any desired level from the 3-D data stored in the computer.     

Doppler Estimation of Pulmonary Artery Systolic Pressure Revisited

Previous attempts at noninvasive estimation of pulmonary artery systolic pressure (PA_S) were limited by the inability to identify adequate spectral signals of tricuspid regurgitation (TR) in a significant proportion of patients. Recently, image and color flow directed continuous-wave Doppler have enabled spectral mapping of previously undetectable TR jets. Accordingly, 30 consecutive patients underwent two-dimensional and continuous-wave Doppler echocardiograms immediately before right heart catheterization. PA_S was calculated as ∑ (4V²+ RA) where Vis the maximal velocity of the TR jet in ml sec and RA is right atrial pressure as estimated by the caval respiratory index. Twenty-eight patients (93%) had detectable signals of TR. Directly measured PA_S ranged from 21 to 95 mmHg (mean ± SD, 40 ± 18). Correlation between Doppler and catheter-measured PA_S was R-sq = 0.93 with a standard error of the estimate of 4.7 mmHg. Accurate results were obtained over a wide range of pulmonary pressures. Thus, noninvasive determination of PA_S is feasible in most patients including those with normal PA_S, and correlates extremely well with direct measurements.     

The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports

Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.     

Unique Echocardiographic Markers of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) in the Adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect in adults. We report a 38‐year‐old male presenting with exertional syncope. He was referred for the evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Echocardiography revealed mild left ventricular enlargement, abnormal flow‐pattern in the ventricular septum and dilatation of the right coronary artery. Pulsed‐wave Doppler with sample volume placed in the coronary ostium showed systolic coronary flow predominancy. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. Coronary angiography confirmed ALCAPA syndrome. Surgical correction was planned.     

Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism

Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease.The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.     

Transesophageal Three-Dimensional Echocardiographic Assessment of Left Main Coronary Artery Fistula

We describe a patient with a left main coronary artery fistula with an opening at the superior vena cava-right atrial junction in whom three-dimensional transesophageal echocardiography permitted more accurate assessment of the shape and size of the communication site than two-dimensional transesophageal echocardiography.     

Assessment of Coronary Artery Fistula by Color Doppler Echocardiography

Two patients with coronary artery fistula (CAF) were studied by color Doppler echocardiography, left coronary artery-right ventricle fistula in a child and right coronary artery-left ventricle fistula in an adult. Diagnosis was made by transthoracic echocardiography (TTE) and one adult by multiplane transesophageal echocardiography (TEE), respectively. The proximal dilated coronary artery, the course, and the drainage sites were demonstrated with color Doppler echocardiography in both patients.     

Color Doppler Transesophageal Echocardiography in Detection of Massive Pulmonary Embolism

In this article, the potential value of color Doppler in improving diagnostic accuracy of transesophageal echocardiography (TEE) in patients with incomplete obstruction of large pulmonary vessels is illustrated, We present an unusual case of massive pulmonary embolism that was unequivocally detected by color Doppler TEE both before and after pulmonary angiography, which failed to demonstrate filling defects in the pulmonary artery.     

Multiplane Transesophageal Echocardiographic Detection and Differential Diagnosis of Isolated Right Pulmonary Artery Agenesis

We present a case report of isolated right pulmonary artery agenesis detected by multiplane trans-esophageal echocardiography (TEE). A special emphasis is placed on its important role in the differential diagnosis and hemodynamic and functional evaluation of patients with unilateral pulmonary oligemia on chest radiograph. We also present a case report of pulmonary embolism with complete obstruction of the right pulmonary artery for better understanding and for comparison of TEE images in patients with pulmonary artery agenesis.     

Pulmonary Artery Sarcoma：A Case Report of Surgical Cure and 1- Year Follow-up

Pulmonary artery sarcoma is a rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. With heightened clinical awareness and advancing technology, the diagnosis is now increasingly being made preoperatively. Previous literature has described the surgical resection as the single most effective modality for short-term disease palliation. We present the case of a patient in whom pulmonary artery sarcoma was diagnosed preoperatively and who underwent surgical resection as main and left pulmonary artery tumor decollement and fight pneumonectomy with extracorporeal circulation. During 1 year follow-up, the patient was doing well without any symptoms in the first 7 months after operation, then recurred after 8 months and died 1 year later. Also it suggests that early identification and aggressive surgical intervention has the possibility to be curative.     

Two-Dimensional Echocardiographic Findings of Pulmonary Valve Cyst Secondary to Pulmonary Valvuloplasty

Real time two-dimensional transthoracic and transesophageal echocardiography demonstrated a mobile echolucent mass attached to the pulmonary valve in a 25-year-old adult, 20 years following balloon pulmonary valvuloplasty. The mass was surgically excised and pathology showed it to be a cyst.     

Echocardiographic Evaluation of External Iliac Artery Doppler Waveform in Patients with Coronary Artery Disease

Visual interpretation of the Doppler waveform in the common femoral or distal external iliac artery (EIA) was reported to be useful in screening for proximal peripheral artery occlusive disease (PAOD) in patients with lower limb ischemia. Commonly patients with coronary artery disease (CAD) referred for echocardiography have coexistent arterial pathology. Therefore, we decided to study whether echocardiographic evaluation of the distal EIA flow can be useful for detection of PAOD in patients with CAD. We studied 150 consecutive patients (pts) with CAD referred for echocardiography. At the end of an echocardiographic examination, evaluation of the flow in the distal EIA with an echocardiographic probe was performed. The Doppler waveform was classified as normal—with early diastolic flow reversal or abnormal–without early diastolic flow reversal. Echocardiographic findings were compared in a blinded fashion with the results of the ankle brachial index measurements (ABI). Based on the ABI ≤ 0.9, peripheral artery disease was diagnosed in 54 pts (36%) and abnormal external iliac Doppler waveform was found in 27 pts (18%). Sensitivity of abnormal external iliac Doppler waveform in predicting PAOD was 48%, specificity 99%, positive predictive value (PPV) 96%, and negative predictive value 77%. Peripheral arterial occlusive disease is common in patients with CAD referred for echocardiographic study. Echocardiographic assessment of distal EIA Doppler waveform has low sensitivity, but high specificity and high PPV in the diagnosis of peripheral arterial occlusive disease.     

Echocardiographic Estimation of Pulmonary Vascular Resistance in Chronic Thromboembolic Pulmonary Hypertension: Utility of Right Heart Doppler Measurements

The ratio of tricuspid regurgitation velocity divided by the velocity‐time integral of right ventricular outflow tract pulsed‐wave Doppler tracing (TRV/VTI_RVOT) has been used to estimate pulmonary vascular resistance (PVR). However, this method has not been validated in chronic thromboembolic pulmonary hypertension (CTEPH). We assessed the utility of TRV/VTI_RVOT in patients with CTEPH and PVR from 2 to 20 WU. All had right heart catheterization (RHC) within 2 days of echocardiography. TRV/VTI_RVOT was calculated and RHC‐derived pressures, PVR, and cardiac outputs were recorded. Mean pulmonary artery pressure was 47 ± 12 mmHg, cardiac output: 4.2 ± 1.1 L/min, PVR: 9 ± 4 WU, right atrial pressure: 12 ± 6 mmHg. Mean VTI_RVOT was 13 ± 5 cm; mean TRV was 4.2 ± 0.8 m/s, mean tricuspid regurgitation severity was 2.5 ± 0.8 (1 = trace, 2 = mild, 3 = moderate, 4 = severe). Regression analysis demonstrated a correlation between RHC PVR and TRV/VTI_RVOT: PVR = 19.4 × (TRV/VTI_RVOT) + 2.4 (r = 0.74, P < 0.001). However, Bland–Altman analysis found a poor degree of agreement between echo‐derived PVR and RHC PVR. We also studied 28 patients with non‐CTEPH pulmonary hypertension. Similar analysis revealed a regression equation of PVR = 20.1 × (TRV/VTI_RVOT) + 0.3 (r = 0.57, P < 0.01). Conclusion: TRV/VTI_RVOT is only marginally useful for estimating PVR in CTEPH (r = 0.74). Moreover, the regression equation in CTEPH differs significantly from previous studies in pulmonary hypertension. Reasons for this may include the markedly elevated PVR levels in this population and specific effects on VTI_RVOT from CTEPH.     

Transesophageal Color Doppler Three-Dimensional Echocardiographic Assessment of Left Circumflex Coronary Artery Fistula

We describe an adult patient with a left circumflex coronary artery fistula in whom color Doppler three-dimensional transesophageal echocardiography demonstrated clearly the exact site of the communication with the coronary sinus near the left atrial appendage. This could not be delineated definitively by multiplane two-dimensional transesophageal echocardiography.