Unilateral Pulmonary Vein Atresia Research Articles

Unilateral pulmonary vein atresia: Difficulties of radiological diagnosis

Pulmonary vein atresia is a rare congenital abnormality that could manifest in isolation or in association with other congenital abnormalities in the cardiovascular system such as pulmonary vein hypoplasia. Pulmonary vein atresia leads to changes in cardiovascular functioning. This abnormality is often diagnosed in children with recurrent pneumonia and hemoptysis. In adulthood, pulmonary vein atresia is much less common, with clinical symptoms such as dyspnea during physical exercises and hemoptysis. However, some patients are asymptomatic. Owing to the nonspecific imaging findings, lung parenchymal changes are often misdiagnosed as another lung disease, including inflammatory genesis disease. In this article, a case of a young man with asymptomatic unilateral pulmonary vein atresia combined with pulmonary artery hypoplasia and interstitial lung changes in a lung with hypoplasia was presented. These pathologies were first identified in a 21-year-old patient by contrast-enhanced computed tomography.

Unilateral pulmonary vein atresia presenting with recurrent hemoptysis and bronchial varices in an Ethiopian adolescent: a case report

BackgroundCongenital unilateral pulmonary vein atresia is a rare anomaly resulting from failure of the pulmonary vein to incorporate in the left atrium. It is a very rare cause of recurrent respiratory infections and hemoptysis requiring a high index of suspicion for proper diagnosis and management in early childhood.Case presentationWe report a 13-year old Anuac (Ethiopia, Region of Gambela) male adolescent with a delayed diagnosis of isolated atresia of the left pulmonary veins despite early childhood presentation with recurrent chest infections, hemoptysis and exercise intolerance. Contrast enhanced CT of thorax with reconstructed planes confirmed the diagnosis. He underwent pneumonectomy for severe and recurrent symptoms and did well on subsequent follow ups after 6 months of pneumonectomy.ConclusionAlthough a rare anomaly, congenital unilateral pulmonary vein atresia should be considered in the differential diagnosis of a child presenting with recurrent chest infections, exercise intolerance and hemoptysis to facilitate early appropriate diagnosis and treatment.

Right Sub-axillary Incision for Right Pulmonary Vein Atresia in a Child: A Case Report and Literature Review

Unilateral pulmonary vein atresia (UPVA) in children is a rare disease that is characterized by a recurrent pulmonary infection and hemoptysis in childhood. This paper is a report of a case of pulmonary venous atresia treated by a right sub-axillary incision. Hopefully, more literature can be produced to improve the awareness and treatment level of pulmonary vein atresia.

379 - Beyond our expectorations: a case of hemoptysis due to unilateral pulmonary vein atresia

379 - Beyond our expectorations: a case of hemoptysis due to unilateral pulmonary vein atresia

Mediastinal and hilar soft tissue mass-like lesions in congenital unilateral pulmonary vein atresia: A retrospective review of seven pediatric patients.

Congenital unilateral pulmonary vein atresia (CUPVA) is known to lead to the formation of an abnormal confluent mediastinal and hilar soft tissue mass, thoracic hypoplasia, and interlobular septal thickening on the affected side. The purpose of the present study is to investigate the frequency and severity of mediastinal soft tissue mass-like lesions and examine other abnormal findings associated with CUPVA. We retrospectively reviewed seven children with CUPVA who underwent contrast-enhanced CT scans and measured the soft tissue mass volume in the bilateral mediastinum (affected and normal side). The location of abnormal soft tissue was divided into three anatomical sections (paratracheal, peribronchial, and the dorsal aspect of the left atrium). The relationships among soft tissue volume and anatomical section were statistically evaluated. Also, the presence of thoracic hypoplasia, small ipsilateral pulmonary arteries, interlobular septal thickening, and ground-glass opacities were investigated. In all cases, CT scans confirmed the presence of confluent soft tissue mass-like lesions in the affected mediastinum. The soft tissue volume on the affected side was 5.5-fold greater than the volume on the normal side (average: 18.0cm3 and 4.25cm3 respectively, P < 0.01). Thoracic hypoplasia and interlobular septal thickening were found in all patients. Small pulmonary arteries and ground-glass opacities were present in six of the seven patients. Abnormal mediastinal and hilar soft tissue is commonly found in patients with CUPVA. So, if we encounter the mediastinal soft tissue mass in patients with CUPVA, no further test will be indicated.

Unilateral pulmonary vein atresia presenting with recurrent haemoptysis in a child: a case report

BackgroundHaemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child.Case presentationA 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation excluded a foreign body aspiration but revealed right bronchial mucosal hyperaemia and varices. Diagnosis of right unilateral PVA was suspected on transthoracic echocardiography which demonstrated hypoplastic right pulmonary artery and non-visualization of right pulmonary veins. Final diagnosis was confirmed on cardiac CT angiography. A conservative treatment approach was opted with consideration for pneumonectomy in future when she is older.ConclusionRarer causes should be considered when investigating for recurrent haemoptysis in children. Bronchoscopy and cardiac imaging are useful tools to establish the diagnosis of unilateral PVA in our case.

Right Pulmonary Vein Atresia in a Mildly Symptomatic Boy: Comprehensive Analysis of Flow Dynamics Using Non-contrast-enhanced 4D Flow MR Imaging

We present multimodal imaging in the rare case of isolated unilateral pulmonary vein atresia in a 6 year-old boy, including analysis of hemodynamics by magnetic resonance acquisition technique of time-resolved three-dimensional phase contrast imaging (4D flow magnetic resonance imaging). This novel imaging method enables the quantification and especially comprehensive visualization of blood flow patterns, even in complex congenital anomalies which abducted detailed assessment so far, and therefore constitutes a promising alternative to conventional vascular imaging techniques.

Proximal Interruption of the Pulmonary Artery and Unilateral Pulmonary Vein Atresia.

Proximal Interruption of the Pulmonary Artery and Unilateral Pulmonary Vein Atresia.

Congenital unilateral pulmonary vein atresia in children: report of two cases

Congenital unilateral pulmonary vein atresia in children: report of two cases

Unilateral pulmonary vein atresia of children: 4 cases report and literature review

Objective To summarize the clinical characteristics, imaging characteristics, treatment and prognosis of unilateral pulmonary vein atresia (UPVA) in children and to improve the clinician′s understanding of this disease. Methods The clinical data of 4 cases of UPVA from January 2014 to December 2016 in Department of Cardiology, Guangzhou Women and Children′s Medical Center were retrospectively analyzed, and 50 cases from reviews of PubMed, OVID and Elsevier in the international medical literature database and 4 cases in Wanfang database for the domestic report were reviewed.The clinical characteristics, diagnosis, treatment and prognosis of total of 58 cases were analyzed. Results Four patients, with an average age of 1.8 years (1.5-2.7 years), showing congenital UPVA in 3 patients and secondary UPVA in 1 patient.There was 1 case of left upper pulmonary vein atresia, 2 cases of left pulmonary vein atresia and 1 case of right pulmonary vein atresia.Three cases showed recurrent hemoptysis and recurrent cough occurred in 1 case.Three cases were complicated with congenital heart disease.There was one case underwent bronchial artery embolization, 1 case received pulmonary vein left atrium connection, and 2 cases received conservative treatment.All patients had been followed up for 1-3 years so far.The patient receiving pulmonary vein left atrium had been completely cured, and the patient receiving bronchial artery embolization showed no occurrence of hemoptysis but still showed recurrent cough hemoptysis.The patient with secondary UPVA had no obvious clinical symptoms, the other 1 case who rejected operation and received conservative treatment still had recurrent pulmonary infection, intermittent hemoptysis.The average age of 54 cases(40 cases with age ≤18 years old) from the literature reports was 13.76 years (8 days -43 years) in which 52 cases were diagnosed as congenital UPVA, while 2 cases were secondary UPVA.Twenty-seven cases were right pulmonary vein atresia, 22 cases were left pulmonary vein atresia, and 5 cases were other types.There were 94.4%(51/54 cases) of the patients having recurrent cough, pulmonary infection, 92.6%(50/54 cases) of the patients with exertional dyspnea and polypnea, 68.5%(37/54 cases) of the patients with hemoptysis and hematemesis.There were 50.0%(27/54 cases) of UPVA patients who were complicated with heart malformation.Different degrees of pulmonary hypertension were observed in 75.9%(41/54 cases) of children, and 35.2%(19/54 cases) of patients had pulmonary lymphatic dilatation.Pulmonary resection was performed in 25 cases, pulmonary vein left atrium connection was performed in 11 cases, bronchial artery embolization was performed in 7 cases, and conservative treatment was performed in 11 cases.After operation, most of the patients had good prognosis without obvious clinical symptoms or mild symptoms. Conclusions In clinical practice, if unexplained hemoptysis, recurrent lower respiratory tract infection, pulmonary consolidation, pulmonary dysplasia or pulmonary hypertension present, the possibility of UPVA should be considered.Early diagnosis and early bronchial artery embolization, reconstruction of the pulmonary vein and atrial connection and repair of the defect of heart, can improve the symptoms or cure the children and reduce the morta-lity significantly. Key words: Child; Pulmonary vein atresia; Unilateral; Hemoptysis; Pneumonia

Unilateral Pulmonary Vein Atresia with Contralateral Upper Pulmonary Vein Atresia in an Adult Patient

Pulmonary vein atresia (PVA) is a very rare anomaly divided into common, individual and unilateral PVA. We report a case of a 54 year-old woman who presented with a history of recurrent dyspnea and pulmonary infections. The diagnosis of PVA was suspected in echocardiography and confirmed with CT angiography which demonstrated absent pulmonary veins except for the left inferior pulmonary vein. Pulmonary arteriography confirmed these findings. In our knowledge, the case of unilateral PVA with contralateral upper pulmonary vein atresia in an adult patient has never been reported in literature.

Isolated unilateral pulmonary vein atresia.

Isolated unilateral pulmonary vein atresia.

Unilateral Pulmonary Vein Atresia Presenting in Adulthood

Unilateral Pulmonary Vein Atresia Presenting in Adulthood

Symptomatic paroxysmal atrial fibrillation in a patient with unilateral pulmonary vein atresia

Symptomatic paroxysmal atrial fibrillation in a patient with unilateral pulmonary vein atresia

Symptomatic paroxysmal atrial fibrillation in a patient with unilateral pulmonary vein atresia

A 52-year-old man with symptomatic paroxysmal atrial fibrillation was offered an atrial fibrillation (AF) ablation procedure. His echocardiogram indicated that he had no structural heart disease. A cardiac computed tomographic (CT) scan showed enlargement of the right pulmonary veins, absence of the left pulmonary veins, a prominent left atrial appendage, and a hypoplastic left lung. Cardiac CT with an electroanatomic mapping system confirmed a prominent left atrial appendage and the absence of the left pulmonary veins. Due to the limited number of patients with this condition, information about ablation remains very limited, and his ablation was deferred. Unilateral pulmonary vein atresia is a rare condition in adults which results from failure of incorporation of the common pulmonary vein into the left atrium. This case demonstrates the clinical importance of preprocedural imaging prior to AF ablation.

Acquired unilateral pulmonary vein atresia in a 3-year-old boy

While unilateral pulmonary venous atresia (UPVA) most commonly presents as an extremely rare late embryological defect resulting in complete occlusion or absence of the PV pathway, it may also be an acquired pathology. We present a 3-year-old boy who presented with mild respiratory distress. Neonatal echocardiographic investigations revealed normal mediastinal anatomy and pulmonary vasculature with a bicuspid aortic valve. However, follow-up Doppler investigation revealed a pulmonary artery size difference with minimal forward flow and reverse flow during diastole. Absence of the left pulmonary veins and the presence of collaterals draining to the innominate vein confirmed the diagnosis of acquired UPVA. Our case represents the first case of acquired UPVA in conjunction with a normally functioning bicuspid aortic valve. The Doppler flow patterns discussed might be of significant interest to pediatricians, cardiologists and imaging specialists. These findings suggest that acquired UPVA should be considered in the differential diagnosis of such patients when radiographic and echocardiographic findings may rule out other more common diagnoses. While the management of such a condition remains unclear and conservative management was agreed upon for our patient, the vulnerability of such cases warrants timely diagnosis and routine monitoring.

Unilateral pulmonary veins atresia: evaluation by computed tomography

Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient.

Bronchial Varices in Congenital Unilateral Pulmonary Vein Atresia

Bronchial Varices in Congenital Unilateral Pulmonary Vein Atresia

Unilateral left pulmonary vein atresia: radiologic findings in an adult case.

An 18-year-old young man who had been physically healthy presented with cough and fever after cold. A nonenhanced chest CT scan showed a small left hemithorax with thickened interlobular lines as well as a mild ipsilateral mediastinal shift (Figure 1). Contrast-enhanced CT angiography revealed a small left pulmonary artery (LPA) with poor contrast enhancement compared with the right pulmonary artery. No pulmonary veins were identified on the left side, but abundant collateral vessels reflecting the mal-distribution of blood flow were noted. Posterior view of volume-rendered image of the LPA found the left border of the left atrium was smooth, without evidence of rudimentary pulmonary veins (Figure 2).

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

Unilateral pulmonary vein atresia is a very rare congenital anomaly associated with high morbidity and mortality. Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis. Further, the diagnosis can often be difficult to make. We present an infant who died due to unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis who was diagnosed with the assistance of cardiac computed tomography scanning.