Pulmonary sequestration in a newborn mimicking cardiac disease: A trap for diagnosis

A case of pulmonary sequestration in a neonate presenting with hyperdynamic circulation and a murmur is reported. Clinical, radiographic and echocardiographic data suggested the diagnosis and this was confirmed by angiography. The surgical findings were those of sequestration of the right lower lobe, in conjunction with an accessory right lung complete with bronchial and vascular supply arising from the region of the lower oesophagus. Pulmonary sequestration may mimic primary cardiovascular disease in the neonate.     

Pulmonary sequestration in infants: diagnosis and therapy exemplified by a newborn infant

Infants with intra- or extralobular lung-sequestrations normally suffer from chronic infections. In our case an infant is described who had a triangular area in chest X-rays behind the heart. Lung scintigraphy and computer-tomography could not commit the diagnosis. Only invasive angiography showed the sequestration. Although without symptoms the baby was operated preventively. Postoperative course was without complications.     

Lung sequestration as a cause of acute cardiac decompensation in a 3-week-old newborn infant

A 3 week old neonate developed congestive heart failure after the operation of an omphalocele. The clinical aspect first suggested to us a persistent ductus arteriosus. Cardiac catheterisation then revealed a bronchopulmonary sequestration, a rare congenital malformation. Children usually become symptomatic with chronic respiratory tract infections, although there are also asymptomatic courses. Only a few cases are reported with congestive heart failure being the initial symptom. Many patients show additional malformations--so did our baby with an omphalocele and a mild peripheral pulmonary stenosis. Once the diagnosis is made, resection or lobectomy is the therapy of choice. In the preoperative diagnosis angiography is mandatory to demonstrate the abnormal vascular supply.     

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.     

Pulmonary varix diagnosed in a newborn

A case of a pulmonary varicose vein is described. A coin lesion was observed and persisted after a pneumonia during the first month of life. An anomalous pulmonary vessel was suspected by tomography, and was verified by selective pulmonary angiography when the infant was three months old.     

Pulmonary veno-occlusive disease: diagnosis during life in four patients

Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension of unknown aetiology. Four cases were diagnosed in young patients. The diagnosis was suspected on the basis of clinical, radiological, echocardiographic, and catheter evidence and confirmed by taking a lung biopsy sample. In all patients the histology showed obstruction of the pulmonary veins by intimal fibrosis. The clinical course of all patients has been one of progressive deterioration. Although there is no specific treatment for this disease, to establish the diagnosis during life is of great importance in overall clinical management, including counselling the patient and family.     

Pulmonary sequestration and diaphragmatic eventration in a 6-month-old infant

A congenital pulmonary malformation, such as pulmonary sequestration or congenital cystic adenomatoid malformation, should be suspected in infants with recurrent lower respiratory symptoms or unifocal infiltrations. The possibility of congenital pulmonary malformation associated with additional abnormalities, such as diaphragmatic hernia, is relatively high and can lead to misdiagnosis. We report a case of a 6-month-old girl who presented with relapsing respiratory infection and hematemesis. Computerized axial tomography scan and barium swallowing study were performed, revealing a suspected intralobar pulmonary sequestration associated with sliding gastric hernia. Since the patient's condition was complicated by sliding hernia, pneumonia and pleural effusion, a surgical procedure instead of cardiac catheterization with coil embolization was performed. During surgery, the absence of a sliding gastric hernia but a diaphragmatic eventration was noted. Only a partial portion of the left-side diaphragm was relaxing, making the barium swallowing study difficult to interpret. This case serves as a reminder that if pulmonary sequestration is suspected, a full work-up with a complete set of imaging studies should be ordered for the possible detection of associated gastrointestinal, respiratory and thoracic abnormalities.     

Upper thoracic extralobar pulmonary sequestration presenting with respiratory distress in a newborn

An upper thoracic extralobar pulmonary sequestration in a newborn is presented. It was present on the initial chest radiograph taken because of respiratory distress. The sequestration was surgically removed and the infant's respiratory difficulties improved.     

Pulmonary hypertension in the newborn

Pulmonary hypertension of the newborn occurs in 1.9 per 1000 live births and affected infants are hypoxaemic because of right-to-left shunts through the ductus arteriosus and foramen ovale. Pulmonary hypertension of the newborn may be primary, or secondary to a variety of conditions including intrapartum asphyxia, infection, pulmonary hypoplasia, congenital heart disease or drug therapy. It may occur in association with a normal number (maladaptation) or a decreased number of arteries (for example with pulmonary hypoplasia). Few strategies used in infants with pulmonary hypertension of the newborn have been subjected to rigorous evaluation. Inhaled nitric oxide has been shown to reduce the need for extracorporeal membrane oxygenation but not mortality, in term or near term born infants. Preliminary evidence suggests that other vasodilators given by the inhaled route may improve oxygenation and new vasodilators have become available; appropriately designed trials with long-term outcomes are required to test such therapies.