Absence Of Right Pulmonary Artery Research Articles

Congenital unilateral absence of right pulmonary artery with VSD and wide aortopulmonary window in an adult patient: a case report.

We present a case of a 28-year-old male who visited our center with the diagnosis of ventricular septal defect with Eisenmenger syndrome elsewhere for further evaluation and was found to have right-sided UAPA with ipsilateral pulmonary hypoplasia and some associated cardiac anomalies. Discussions are held regarding typical chest radiograph findings, diagnostic methods, and possible therapies. Physicians should be aware of UAPA, which might go undiagnosed for several years despite regular medical care and can show up later in life, causing chronic respiratory symptoms along with Eisenmenger syndrome and ventricular septal defect like in our case.

An isolated congenital absence of right pulmonary artery associated with pulmonary hypertension with coronary collaterals: A case report

Unilateral absence of pulmonary artery (UAPA) resulting from a defect in the aortic arch's embryogenesis. Patients who survive into adulthood is rare. Herein, we presented a case of UAPA with pulmonary hypertension in a 49-year old female.

Unusual association of isolation of right pulmonary artery in absent pulmonary valve syndrome.

Absence of arterial duct, a sixth aortic arch derivative, plays an important etiologic role in Tetralogy of Fallot with absent pulmonary valve syndrome. When fetal ductus is absent, the large right ventricular stroke volume dilates the pulmonary trunk leading to pulmonary regurgitation. A proximal extension of the embryonic insult to the entire left sixth arch causes absence of the left pulmonary artery, a common association of absent pulmonary valve syndrome. On the contrary, absence of right pulmonary artery is not reported in absent pulmonary valve syndrome. A rare combination of tetralogy, absent pulmonary valve syndrome and isolation of a hypoplastic right pulmonary artery offered challenges to diagnosis and management.

Infantile Isolated Absence of Right Pulmonary Artery with an Absent Stump and No Collaterals

AbstractUnilateral absence of pulmonary artery (UAPA) is a rare entity. Diagnosis is often incidental based on echocardiography (ECHO)/computed tomography (CT) findings done for a cardiac evaluation or recurrent pulmonary pathology not responding to conventional treatment. Childhood-onset symptoms are often rare, and most of these cases are reported in adulthood or postpuberty. Here, we present a 5-month-old child with an isolated absence of right pulmonary artery without even an arterial stump or collaterals. Treatment options depend on the severity of symptoms and anatomy of the abnormal vessels, and in some cases, patients get better, if not completely well with the available medical and surgical treatments.

Unilateral absence of pulmonary artery analysis based on echocardiographic feature.

The unilateral absence of pulmonary artery (UAPA) is a rare congenital cardiovascular malformation, which is asymptomatic and easy to be ignored in early stage. A large number of complications may occur in the later stage. Therefore, early diagnosis and treatment is of great significance. The imaging data of 49 patients with UAPA discovered and confirmed clinically by the echocardiography in our hospital are analyzed. The results show that left pulmonary artery absence is more common (55%) and most of them are associated with other cardiovascular malformations (92%). Atrial septal defect and patent foramen ovale were most common in 56%. In which the absence of isolated pulmonary artery was 8% (4/49), and the absence of right pulmonary artery was 75% (3/4). Especially in the patients with tetralogy of Fallot, 77% (5/9) of them miss the diagnosis of UAPA. This suggests that doctors and sonographers should pay attention to the development of pulmonary artery bifurcation and left and right branches in multi-section, and strengthen the scanning of short axis section of high large artery.

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta and unilateral absence of pulmonary artery by echocardiography

Objective To improve the prenatal diagnosis and differential diagnosis ability for anomalous origin of one pulmonary artery branch from the aorta (AOPA) and unilateral absence of pulmonary artery (UAPA) by comparing and analyzing different types of fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in fetus with prenatal diagnosis of UAPA or AOPA between June 2012 and December 2018, and the different types of fetal echocardiography characteristics and main points of identification were summarized. Results A total of 6 fetuses with AOPA and 6 fetuses with UAPA were diagnosed.Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and 1 case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with an absent pulmonary valve. All 6 cases of AOPA had proximal form. Among the 6 fetuses with UAPA, 4 cases were isolated unilateral absence of right pulmonary artery, 1 case was isolated unilateral absence of left pulmonary artery and 1 case was unilateral absence of left pulmonary artery combined with tetralogy of Fallot (TOF). The main common characteristic sonographical findings included: there was no confluence at the bifurcation of the main pulmonary artery on multiple views, and the affected pulmonary branch was not attached to the main pulmonary artery. The different characteristic sonographical findings in fetus included: the affected pulmonary artery of AOPA arosed from the right posterior or left posterior portion of the ascending aorta on three vessels and trachea view (3VT) and long axial view of left ventricle, and 3VT and the coronary view of brachiocephalic trunk artery showed that the abnormal origin of pulmonary artery branch of UAPA connected with the root of brachiocephalic trunk artery by the ipsilateral vertical arterial duct (DA), and color Doppler flow imaging showed that the blood flow of the affected pulmonary artery branch was isolated in the pulmonary field. Conclusions AOPA and UAPA have many similarities in the characteristics of fetal echocardiography. 3VT and long axial view of left ventricle are the best views for displaying proximal AOPA, 3VT and the coronary views of the root of brachiocephalic trunk artery are the best views for displaying UAPA. Key words: Echocardiography; Fetus; Unilateral absence of pulmonary artery; Anomalous origin of one pulmonary artery branch from the aorta

Unusual presentation of rare congenital anomaly: Isolated unilateral absence of a pulmonary artery

Unilateral absence of a pulmonary artery (UAPA) is a rare defect. It may be associated with other congenital cardiovascular malformations which present at an early age. Isolated UAPA (IUAPA), i.e., without any other cardiovascular malformations, usually is asymptomatic and presents in adulthood. In infancy, IUAPA may be suspected by the presence of recurrent respiratory infections and pulmonary hypertension (PHT). Here, we present a child with IUAPA who presented with intermittent cyanosis which is unusual. Echocardiography done showed the presence of severe PHT with a patent foramen ovale (PFO) with a right to left shunt. Detailed echo revealed the absence of right pulmonary artery which was confirmed by a multislice detector computed tomography (MDCT). Infants with unexplained PHT should be screened for the possibility of IUAPA. MDCT scan and magnetic resonance imaging (MRI) can confirm the echocardiographic diagnosis. The treatment plan depends on the presence of symptoms, size of pulmonary artery, and collaterals. Early surgical or hybrid intervention may improve survival. Medical management includes treatment for congestive cardiac failure and pulmonary vasodilators for PHT. Infants with severe PHT are difficult to treat and have poor prognosis.

A Case Series of Young Patients with Completely Reversed Severe Pulmonary Hypertension

Pulmonary Arterial Hypertension (PAH) is a progressive symptomatic disorder, which may ultimately lead to death if left untreated. Although majority of PAH cases are idiopathic, pulmonary hypertension resulting due to certain underlying conditions are also observed frequently. In such cases, it becomes essential to identify any potentially treatable or reversible causes for PAH. There have been significant advances in the medical management of PAH and various medicines have been approved by US Food and Drug Administration (FDA) for various stages of PAH. With these therapies, there can be varying degrees of improvement in the pulmonary artery pressures and hemodynamic profile. Therefore, physiologic reversal can and does occur, sometimes to the point of normalization. We hereby present three such cases of severe PAH in patients below 50 years of age due to various aetiologies like left heart disease, isolated unilateral absence of right pulmonary artery with hypoplastic right lung and factor V Leiden mutation associated pulmonary thromboembolism, all of whose pulmonary artery pressures are completely normalised with adequate treatment of the underlying disease and with optimised medications for PAH, ultimately leading to tapering and stoppage of PAH medications.

Repair of Unilateral Absence of Right Pulmonary Artery With Contralateral Pulmonary Artery Autograft Interposition in an Infant

Unilateral absence of the pulmonary artery (PA) is a rare congenital abnormality. Although a traditional 2-stage approach with a preceding systemic-to-PA shunt followed by a definitive repair is mandatory in patients with absent extrapericardial pulmonary arteries, more creative solutions are necessary when 1-stage repair is concerned. We present anovel technique for repairing the absent intrapericardial right pulmonary artery (RPA) with a contralateral PA autograft segment interposition. We believe that this technique using solely autologous tissue is effective in patients in whom the intrapericardial segment of the PAs is absent.

Case report of isolated congenital absence of right pulmonary artery with collaterals from coronary circulation

Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital lesion which is often associated with other cardiovascular abnormalities and a diverse clinical presentation. It is usually diagnosed in childhood. Patients who survive into adulthood is uncommon. We report a case of 46 year old hypertensive and obese female who presented with progressive dyspnea. She had features of pulmonary hypertension. The diagnosis was confirmed by CT pulmonary angiography which showed absence of right pulmonary artery and conventional pulmonary angiography which showed ipsilateral lung receiving collaterals from Right coronary artery and its branches. The purpose of this report is to highlight the fact that UAPA, although a rare entity, should be kept in mind in patients with unexplained PAH and prolonged respiratory symptoms unresponsive to routine treatment modalities.

乳児期早期に二期的再建を行った右肺動脈近位部欠損症の1治験例

乳児期早期に二期的再建を行った右肺動脈近位部欠損症の1治験例

Congenital absence of right pulmonary artery associated with left patent ductus arteriosus and aortic coarctation

Congenital absence of right pulmonary artery associated with left patent ductus arteriosus and aortic coarctation

Proximal interruption of the pulmonary artery

A 37-year-old male presented with hemoptysis for 1 week. His previous chest X-ray (Figure 1A) showed small volume of the right lung, absence of right pulmonary hilum and mediastinum shifted to right. Contrast-enhanced chest CT (Figure 1B) revealed absence of right pulmonary artery and mosaic perfusion (Figure 1C) during expiratory phase. Based on the image findings, the patient received a diagnosis of interruption of right pulmonary artery. Figure 1 ( A ) Chest X-ray shows small volume of the right …

Isolated absence of right pulmonary artery: Radiographic and multi-detector CT demonstration

Isolated absence of right pulmonary artery: Radiographic and multi-detector CT demonstration

Erratum to: Surgical correction of isolated unilateral absence of right pulmonary artery

Erratum to: Surgical correction of isolated unilateral absence of right pulmonary artery

Vascular Changes in Agenesis of Pulmonary Artery

A 25-year-old woman presented to the emergency room with hemoptysis. Her history within the past 5 years included two hospitalizations due to hemoptysis and lung infection, respectively. The magnetic resonance angiographic scan and computerized tomographic scan demonstrated a smaller right lung with multiple blebs and bronchiectasis, absence of right pulmonary artery (Fig 1A), and hypertrophy and tortuosity of the bronchial artery, originating from the descending aorta. The orifice of the bronchial artery measured 6.9 mm in diameter (Fig 1B).

Surgical correction of isolated unilateral absence of right pulmonary artery

A 2-year-old girl with isolated unilateral absence of right pulmonary artery is described. Catheterization at 5 months demonstrated hypoplastic right pulmonary artery by pulmonary venous wedge angiography, and the patient underwent right Blalock-Taussig shunt and angioplasty of right pulmonary artery with autologous pericardial roll as an initial step. At 2 years, she underwent anastomosis of right pulmonary artery to main pulmonary artery with an autologous pericardial tube. Postoperative computed tomography showed a patent reconstructed right pulmonary artery.

Isolated absence of right pulmonary artery

Background:Absence of right pulmonary artery (RPA) is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infantsObjective:The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature.Methods:The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectivelyResults:Five patients were identified with this anomaly. The median age of presentation was 86 days (range, 40–120 days) and the median weight was 3.65 kg (range, 3.1–5.5 kg). All patients presented with breathing difficulty and had severe pulmonary hypertension (PHT) along with absent RPA on echocardiography. A multidetector computed tomographic scan was performed to confirm the diagnosis in four cases. Three patients had major aortopulmonary collateral and the hilar RPA was not well developed in all. A correct diagnosis was made before referral in one patient only. Differential vascularity in chest X-ray, a useful clue, was seen in four of five cases. Surgical correction was not considered in view of the small hilar pulmonary artery. The patients were all managed medically with diuretics and Sildenafil.Conclusion:Isolated absence of RPA is a rare congenital abnormality with varied presentation. Infantile presentation is marked with congestive cardiac failure and PHT. Specific diagnostic clue includes differential vascularity on the chest X-ray.

A Case of Isolated Congenital Absence of Right Pulmonary Artery

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

Three-dimensional reconstruction of right lung circulation in a patient with isolated absence of right pulmonary artery

A 51-year-old man was referred to our hospital for recurrences of palpitation due to atypical atrial flutter. The patient reported several episodes of hemoptysis only during the childhood. No other symptom was present. Chest plain radiograph showed a mild displacement of the heart to the right while only a mild enlargement of both atria was observed by echocardiography. Axial images and three-dimensional reconstructions (volume rendering) of lung and mediastinum using 16-slices Multidetector computed …