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

BACKGROUND: Haemoptysis 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 PRESENTATION: A 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. CONCLUSION: Rarer 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.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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: 18.0 cm3 and 4.25 cm3 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. CONCLUSION: 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.

Comparison of prenatal ultrasound and postmortem findings in fetuses with common pulmonary vein atresia.

BACKGROUND: The previous literature on common pulmonary vein atresia (CPVA) mainly consists of neonatal case reports. There is a lack of research on the prenatal diagnosis of CPVA. METHODS: We conducted a retrospective study of all fetuses with CPVA confirmed by autopsy between August 2010 and May 2018. Prenatal echocardiographic features, autopsy findings, and genetic test results were analyzed. We compared fetal CPVA with total anomalous pulmonary venous return (TAPVR) and neonatal CPVA. RESULTS: During the study period, fetal echocardiography was performed on 31 617 fetuses. Six cases of CPVA were identified by autopsies, including 1 case performed with a cardiovascular cast. All 6 cases (100%) had asplenia syndrome (AS) and bilateral superior vena cava (BSVC). In 1 case (16.7%), the prenatal ultrasound results were in complete agreement with the postmortem findings. Four cases (66.7%) were misdiagnosed as TAPVR by prenatal echocardiography. For the remaining case (16.7%), no pulmonary venous anomalies were detected on prenatal echocardiography. No aneuploidy was identified in any of the cases. There were no statistically significant differences among the proportions of associated complex anomalies and AS between the fetal CPVA and TAPVR groups. The proportion of associated complex anomalies and AS in the fetal CPVA group was higher than that in the neonatal group (P < 0.05). CONCLUSIONS: Prenatal diagnosis of fetal CPVA is difficult and challenging even for experts. Our study showed that fetal CPVA is often combined with AS, complex cardiac malformations, and BSVC. These findings may help in the diagnosis of fetal CPVA.

Common pulmonary vein atresia: report of three cases and review of the literature.

Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.

A Very Rare Case of Co-Existence of Cor Triatriatum Sinister and Left Pulmonary Vein Atresia.

Cor triatriatum sinister (CTS) is a rare congenital abnormality. Clinical presentation of patients with CTS mainly depends on the anatomic features of membrane and may vary from mild or moderate symptoms mimicking mitral stenosis to more severe and complicated cardioembolic stroke or a new onset heart failure. We herein have reported on a young male who presented with the signs and symptoms of mitral stenosis and was diagnosed as CTS with gradient on the orifice of the membrane after transthrocacic echocardiography. Computerized tomographic angiography revealed that the patient had unilateral, left sided pulmonary arterial hypoplasia and pulmonary vein atresia, with only the right pulmonary veins draining into the left atrial chamber. Further cardiac imaging by either computed tomography or magnetic resonance imaging is necessary in order to seek accompanying cardiac and vascular abnormalities. Patients with CTS have improved short and long term survival rates if CTS and accompanying abnormalities are surgically treated before the disease is complicated with heart failure, pulmonary hypertension, stroke and etc.

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

BACKGROUND: Congenital 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 PRESENTATION: We 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. CONCLUSION: Although 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.

Congenital unilateral pulmonary vein atresia: A rare disease with successful outcome in a child.

Unilateral pulmonary vein atresia is a rare congenital anomaly that may occur in association with other congenital heart diseases. We report a rare case of left-sided pulmonary vein atresia in a 2-year-old child who presented with recurrent hemoptysis and was managed surgically with left pneumonectomy.

Unilateral pulmonary vein atresia: Literature overview and case report.

The unilateral absence of the pulmonary vein, known as pulmonary vein atresia, is a rare type of unilateral pulmonary venous hypoplasia caused by the congenital atrophy of the long pulmonary vein segments in one lung. The involved lung may be normal in size or present with hypoplasia and is often characterized by increased interstitial attenuation and interlobular septal thickening due to venous stasis, edema, and fibrosis. Pulmonary angiography often reveals a reduced size for the lateral pulmonary artery, peripherally sparse pulmonary vessels, contrast stasis, and the inability to visualize pulmonary veins. Symptoms include coughing up blood and infection. We present the clinical case of a patient who was initially diagnosed with recurrent hemoptysis due to pulmonary tuberculosis, followed by unsuccessful treatment. Imaging by 64-slice computed tomography with contrast injection using multiplanar reformation and volume rendering techniques allowed this case to be definitively diagnosed. This report emphasizes the epidemiological factors and clinical and imaging features of unilateral pulmonary vein atresia to prevent confusion and facilitate proper diagnosis in similar cases.

Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis.

(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0-4 = no disease-atresia) and lung segments (0-3 = unaffected-severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.

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.

Left hypoplastic lung and hemoptysis-rare familial unilateral pulmonary vein atresia.

Unilateral pulmonary vein atresia (UPVA) is a rare congenital vascular malformation with obliteration of the pulmonary vein. We present a case series of three siblings with variable presentation of UPVA. We suggest a dominant genetic cause based on different paternity. Identifying genetic etiology would contribute to early diagnosis and screening.

Unilateral pulmonary vein atresia without anomalous connection in adult patient with recurrent severe hemoptysis.

Isolated unilateral pulmonary vein atresia (UPVA) is a rare congenital malformation and the management remains controversial. In adults, pneumonectomy is the treatment of choice when significant hemoptysis becomes life-threatening. We report a case of a 28-year-old male with isolated unilateral right atresia of the pulmonary vein who had life-threatening hemoptysis treated with bronchial arteries embolization followed by successful right pneumonectomy.

Unilateral pulmonary vein atresia: A rare case of hemoptysis.

We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia. Isolated pulmonary vein atresia is a rare condition in which patients typically acquire a diagnosis in infancy and early childhood [Mataciunas et al.; Pourmoghadam et al.]. Our patient presented during puberty with several previous episodes of hemoptysis prior to her admission and diagnosis. The initial diagnosis was suspected in our patient from chest computerized tomography (CT), and confirmed with cardiac catheterization and pulmonary angiography. Treatment aim is to preserve lung function and minimize irreversible pulmonary remodeling [Pourmoghadam et al.; Harrison et al.]. Conservative monitoring can be considered with milder or asymptomatic cases, while others may require preoperative collateral artery banding, surgical anastomosis between the pulmonary vein (PV) & left atrium (LA) and even pneumonectomy [Pourmoghadam et al.].

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.

Specific Echocardiographic Findings Useful for the Diagnosis of Common Pulmonary Vein Atresia.

In this paper, we report a case of common pulmonary vein atresia, which is a very rare disease characterized by cyanosis, heart failure and pulmonary hypertension. Reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole detected by echocardiography were found to be specific features useful in diagnosing the disease.

Individual pulmonary vein atresia in adults: report of two cases.

We present two cases of individual pulmonary vein atresia without vestige of an involved pulmonary vein. On CT, we noted the absence or interruption of normal pulmonary venous structures, and the presence of abnormal vascular structures that represented collaterals for the involved lung parenchyma. On angiography, the atretic pulmonary vein was found to drain into the other ipsilateral pulmonary veins through the collaterals.