Absence Of Pulmonary Artery Research Articles

Evaluation of an engineered vascular graft exhibiting somatic growth in lambs to model repair of absent pulmonary artery branch

BackgroundGrowth is the holy grail of tissue implants in pediatrics. No vascular graft currently in use for surgical repairs of congenital heart defects has somatic growth capacity.MethodsBiologically-engineered grafts (6 mm) grown from donor ovine fibroblasts in a sacrificial fibrin gel were implanted into the left pulmonary branch of 3-month old lambs for 3, 6, and 18 months. A control group of Propaten® PTFE grafts was implanted for 6 months.ResultsThe engineered grafts exhibit extensive site-appropriate recellularization after only 3 months and near-normal increase of diameter from the preimplant value of 6 mm to 12.9 mm and also a doubling of length from 6.0 mm to 13.0 mm at 6 months (n = 3) associated with apparent somatic graft growth (collagen content increase of 265% over 18-month, n = 2), along with excellent hemodynamics and no calcification, in contrast to the Propaten® grafts. The left-right flow distribution is nearly 50–50 for the engineered grafts at 6 months (n = 3) compared to about 20–80 for the Propaten® grafts (n = 3), which have less than one-half the diameter, a 6-fold higher pressure gradient, and stunted vascular development downstream of the graft. The engineered grafts exhibit a stable diameter over months 12–18 when the lambs become adult sheep (n = 2).ConclusionsThis study supports the use of these regenerative grafts with somatic growth capacity for clinical trial in patients born with a unilateral absent pulmonary artery branch, and it shows their potential for improving development of the downstream pulmonary vasculature.

CONGENITAL ABSENCE OF PULMONARY ARTERY

Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital condition with a prevalence of 1 in 200,000 (1). The first case was reported in 1868, and to date, 350 cases have been reported (2). It usually occurs in conjunction with other cardiovascular anomalies such as tetralogy of Fallot, VSD, truncus arteriosus, etc. (2), and is typically diagnosed in childhood. Survival into adulthood is rare. Symptoms of this condition can include shortness of breath, chest pain, hemoptysis, reduced exercise tolerance, pleural effusion, and recurrent infections. We encountered a case of a 22-year-old female presenting with these symptoms. Initially, the symptoms suggested pulmonary embolism. However, further investigation through CTPA and Echocardiography revealed the absence of the left pulmonary artery. This case report emphasizes that patients showing signs and symptoms indicative of PAH should also be evaluated for the absence of a pulmonary artery." The pulmonary arteries originate from the sixth aortic arch during the sixteenth week of normal embryonic development. The proximal segment of the 6th aortic arch forms the initial portion of the right & left pulmonary arteries, respectively. The link on the arch's right side vanishes, whereas it establishes a connection through the ductus arteriosus on the left side. Pulmonary artery agenesis arises from the aberrant rotation of the afflicted segment's attachment to the aortic arch and dysfunctions during migration (3). Certain publications utilize the phrase “proximal blockage of the pulmonary artery” instead of “agenesis” to describe this anomaly. Although the intraparenchymal distal segment of the pulmonary artery is miniature, it is safeguarded (4). The prevalence of bronchopneumonia is ascribed to multiple causes, such as diminished lung tissue perfusion, impaired mucosal defense, and a possible unbalance of protease-antiprotease enzymes. Moreover, increased collateral blood flow may be associated with the onset of hemoptysis.

A silent anomaly: congenital unilateral absence of pulmonary artery incidentally discovered in adulthood, presenting as pulmonary hypertension and chronic respiratory failure—case report

A silent anomaly: congenital unilateral absence of pulmonary artery incidentally discovered in adulthood, presenting as pulmonary hypertension and chronic respiratory failure—case report

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review.

Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation associated with hemoptysis, pulmonary hypertension, and infection. Little is known about the impact on pregnancy outcomes. We sought to synthesize the existing literature on pregnancy outcomes in patients with maternal UAPA. We report a case of maternal UAPA and performed a systematic review of the existing literature. Articles in English reporting pregnancy outcomes among women with unilateral absence or hypoplasia of the pulmonary artery were included. Articles were reviewed at the abstract level and, if eligible, at the full-text level by two independent reviewers with disagreements adjudicated by a third reviewer. Data were abstracted by two independent reviewers. Outcomes of interest were mode of delivery, gestational age at delivery, intensive care admission, maternal death, and length of stay. Summary statistics for each outcome are presented. We identified 14 studies, including the presented case, reporting outcomes in 22 pregnancies impacted by maternal UAPA. Median age at diagnosis was 21 years. Concurrent cardiac comorbidities were reported in 6/13 (46.2%) with pulmonary hypertension in 5/20 (25%) of cases where this information was reported. We observed high rates of preterm birth (4/12, 33.3%), cesarean delivery (10/15, 66.7%), and operative vaginal delivery (2/5, 40.0%). There was one maternal death occurring in the immediate postpartum period for a mortality rate of 4.5%. Our study provides a comprehensive review of existing literature on maternal UAPA. Our findings suggest increased rates of adverse outcomes and underscore the importance of early diagnosis, identification of pulmonary hypertension, and multidisciplinary care. · There may be increased adverse outcomes in maternal UAPA.. · Concurrent cardiac abnormalities are common in maternal UAPA.. · Early diagnosis, identification of pulmonary hypertension, and multidisciplinary care are important..

Resection of infected bulla associated with isolated unilateral absence of pulmonary artery: a case report

BackgroundA unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly, which in most patients is associated with congenital cardiovascular abnormalities. Some patients with UAPAs do not have associated cardiovascular abnormalities, called isolated UAPA. Patients with isolated UAPA generally have a mild clinical course and may not be diagnosed until adulthood, but repeated complications such as infections may make surgery difficult.Case presentationA 34-year-old female patient with isolated right UAPA was referred to our hospital with a complaint of fever. Chest radiography and computed tomography showed an infectious cavity in the right upper lung. Surgery was scheduled for the patient; however, her preoperative condition needed to be optimized. She was evaluated for pulmonary hypertension and the collateral arteries were mapped out, and a waiting period after treatment of infection was set to avoid perioperative intrathoracic infections. Resection of the infected bulla was performed without any complications. No recurrence of infection was observed two years post-operatively. The residual lung expanded enough to fill the dead intrathoracic space.ConclusionsThis report suggests the importance of adequate preoperative evaluation and perioperative management for patients with isolated UAPA who require surgical resection.

Retroaortic Innominate Vein in Association With Pulmonary Atresia/Major Aortopulmonary Collaterals

BackgroundRetroaortic innominate vein (RAIV) is an extremely rare anomaly of systemic venous return. The prevalence of RAIV has been estimated to be 0.02% in individuals without congenital heart disease and 0.5% in those with congenital heart disease. Previous studies have demonstrated an association between RAIV and both conotruncal abnormalities and right aortic arch. Our center specializes in surgical procedures for patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), and we have frequently observed the presence of RAIV in these patients. The purpose of this study was to evaluate the prevalence and anatomic characteristics of RAIV. MethodsThis was a single-center retrospective review of medical records to identify patients with an RAIV. The study period was 2002 through 2022, with approximately 20,000 patients evaluated during this time frame. ResultsA total of 99 patients were identified with an RAIV. Of the 99 patients, 64 (65%) had PA/VSD/MAPCAs. Other diagnoses included tetralogy of Fallot (n = 16), tetralogy of Fallot with pulmonary atresia (n = 3), double outlet right ventricle (n = 4), peripheral pulmonary artery stenosis (n = 3), and other (n = 9). In the cohort of patients with PA/VSD/MAPCAs, 69% had a right aortic arch and 30% had completely absent central pulmonary arteries. ConclusionsThe overwhelming majority (90%) of patients with RAIV had conotruncal abnormalities, with PA/VSD/MAPCAs accounting for the preponderance of cases in our center.

Isolated unilateral absence of pulmonary artery- an unusual cause of hemoptysis: a case report

BackgroundIsolated unilateral absence of one pulmonary artery (IUAPA) is a rare malformation and a rare cause of hemoptysis. It occurs due to malformation of the sixth aortic arch during embryogenesis. This condition has variable presentations like dyspnoea, reduced exercise tolerance, chest pain, pleural effusion, recurrent bronchopneumonia, pulmonary hypertension, and haemoptysis can be asymptomatic.Case PresentationWe describe a case of a 34-year-old female presenting with recent onset hemoptysis associated with mild dyspnoea and cough. Chest Radiograph revealed unilateral hyperlucent lung field which was later diagnosed as Isolated unilateral absence of pulmonary artery on high resolution computed tomography of chest with angiography.ConclusionAbsent pulmonary artery should be considered as an important differential in cases presenting with hemoptysis. This case emphasises the significance of combining computed tomography with pulmonary angiography to accurately evaluate and define congenital lung abnormalities.

Outcome of patients with tetralogy of Fallot with pulmonary atresia.

Tetralogy of Fallot patients with pulmonary atresia (TOFPA) have a largely varying source of pulmonary perfusion with often hypoplastic and even absent central pulmonary arteries. A retrospective single center study was undertaken to assess outcome of these patients regarding type of surgical procedures, long-term mortality, achievement of VSD closure and analysis of postoperative interventions. 76 consecutive patients with TOFPA operated between 01.01.2003 and 31.12.2019 are included in this single center study. Patients with ductus dependent pulmonary circulation underwent primary single stage full correction including VSD closure and right ventricular to pulmonary conduit implantation (RVPAC) or transanular patch reconstruction. Children with hypoplastic pulmonary arteries and MAPCAs without double supply were predominantly treated by unifocalization and RVPAC implantation. The follow up period ranges between 0 and 16,5 years. 31 patients (41%) underwent single stage full correction at a median age of 12 days, 15 patients could be treated by a transanular patch. 30 days mortality rate in this group was 6%. In the remaining 45 patients the VSD could not be closed during their first surgery which was performed at a median age of 89 days. A VSD closure was achieved later in 64% of these patients after median 178 days. 30 days mortality rate after the first surgery was 13% in this group. The estimated 10-year-survival rate after the first surgery is 80,5% ± 4,7% showing no significant difference between the groups with and without MAPCAs (p > 0,999). Median intervention-free interval (surgery and transcatheter intervention) after VSD closure was 1,7 ± 0,5 years [95% CI: 0,7-2,8 years]. A VSD closure could be achieved in 79% of the total cohort. In patients without MAPCAs this was possible at a significant earlier age (p < 0,01). Although patients without MAPCAs predominantly underwent single stage full correction at newborn age, the overall mortality rate and the interval until reintervention after VSD closure did not show significant differences between the two groups with and without MAPCAs. The high rate of proven genetic abnormalities (40%) with non-cardiac malformations did also pay its tribute to impaired life expectancy.

A Technique for Pulmonary Artery Reconstruction for Ductal Origin of the Right Pulmonary Artery

Ductal origin of the pulmonary artery is a rare cardiovascular anomaly; it is often mislabeled as absent pulmonary artery or pulmonary artery agenesis resulting in delayed interventions. We present a case of ductal origin of the right pulmonary artery highlighting the need for early detection and prompt intervention to reestablish perfusion to the lung. This approach allows for continued lung development and reduces the morbidity related to single lung physiology. The aim of this article is to describe a refinement in the operative technique used in the reconstruction of the ductal origin of the right pulmonary artery via creation of an interposition homograft roll for centralization. Ductal origin of the pulmonary artery is a rare cardiovascular anomaly; it is often mislabeled as absent pulmonary artery or pulmonary artery agenesis resulting in delayed interventions. We present a case of ductal origin of the right pulmonary artery highlighting the need for early detection and prompt intervention to reestablish perfusion to the lung. This approach allows for continued lung development and reduces the morbidity related to single lung physiology. The aim of this article is to describe a refinement in the operative technique used in the reconstruction of the ductal origin of the right pulmonary artery via creation of an interposition homograft roll for centralization.

Pulmonary vein wedge angiography in a child with unilateral absence of pulmonary artery.

A 3-month-old boy was admitted to Hainan Women and Children's Medical Center on November 17, 2019, because of feeding difficulty, without a history of cyanosis, anemia, edema, oliguria, or chronic cardiac diseases, as well as a family history of cardiac diseases or other disorders. Physical examination revealed a systolic murmur of grade II at the left ventricular apex. Laboratory findings showed no abnormalities in either the serum creatine kinase or the serum creatine kinase isoenzyme and anemia, with arterial oxygen partial pressure (PaO2) of 71 mmHg and arterial oxygen saturation (SaO2) of 94%.

Absent Pulmonary Artery Presenting as High-Altitude Pulmonary Edema.

A 22-year-old male with no known past medical history presented to our emergency department complaining of difficulty breathing. A plain film chest radiograph revealed findings consistent with a tension pneumothorax. However, due to physical examination findings inconsistent with the imaging report, a computed tomography of the chest was ordered which revealed an absent right pulmonary artery.The patient was ultimately treated for high altitude pulmonary edema and discharged on nifedipine and supplementary oxygen.

Surgical repair of unilateral absence of pulmonary artery in children with pulmonary hypertension: a single-center retrospective study.

The early diagnosis of unilateral absence of pulmonary artery (UAPA) in children offers an opportunity for effective intervention. Due to the lack of clinical evidence, a consensus regarding surgical treatment has yet to be reported. The aim of this study is to evaluate the effectiveness and safety of pulmonary artery (PA) reconstruction with a "two-segment" technique to repair UAPA in patients with pulmonary hypertension. Intraoperatively, the ligamentum arteriosum connecting the innominate artery and distal PA was dissected and occluded. A conduit created by fresh autologous pericardium formed the first "segment" of the neo-PA. The second "segment" was a Gore vascular graft with integrated rings anastomosed between the proximal end of the pericardial conduit and the main pulmonary artery (MPA). A total of five consecutive patients were included, and the absent PA was successfully reconstructed using the "two-segment" technique in all patients. Following revascularization, the direct measurement of the pressure in MPA during the operation showed that the average mean pulmonary artery pressure (mPAP) decreased from 31.3±16.0 to 16.8±4.2 mmHg (P=0.047). The average mPAP/radial mean arterial pressure (rMAP) ratio decreased from 0.59±0.27 preoperatively to 0.30±0.10 postoperatively (P=0.028). The mean follow-up period was 18.85±4.67 months. The median diameter of the reconstructed PA (pericardial segment) measured by transthoracic echocardiography (TTE) was 6.1 mm. One patient safely underwent a redo operation to repair relative stenosis in the neo-PA. Early PA reconstruction may effectively alleviate pulmonary hypertension in children with UAPA. The "two-segment" technique is safe and can facilitate potential redo pulmonary arterioplasty. Anticoagulation and antiplatelet therapy, as well as frequent follow-up, is required after the operation.

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

ANATOMICALLY SUSTAINABLE VARIATION OF UNILATERAL PULMONARY ARTERY AGENESIS (UPAA)

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.

Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot.

The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.

Monosomy 18p and trisomy 17q in a boy with unilateral absence of the right pulmonary artery

Unilateral absence of the pulmonary artery (UAPA) is an infrequent finding, with an estimated prevalence of 1 in 200,000 young adults. The percentage of syndromic UAPA is not known but mostly does not have a syndromic association, only being reported with the 22q11 deletion syndrome.

Isolated Right Pulmonary Artery Agenesis with Agenesis of Right Upper Lobe and Bronchiectasis of Right Lower Lobe with Anomalous Arterial Supply from Celiac Axis with Normal Venous Drainage

Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

Trans-arterial Embolization in a Patient with Unilateral Absence of Pulmonary Artery: Treatment Success and the Four-year Prognosis.

Unilateral absence of the pulmonary artery (UAPA) with or without other anomalies in the heart is a rare congenital malformation. A 55-year-old Filipino woman without a remarkable medical history was admitted to our hospital for hemoptysis. Contrast-enhanced chest computed tomography revealed the absence of the left pulmonary artery. Echocardiography and right heart catheterization showed no cardiac malformations or pulmonary hypertension. We diagnosed her with isolated left-sided UAPA and performed transarterial embolization of the left inferior phrenic artery. This resolved the hemoptysis, and there was no recurrence during the four-year follow-up period.

Rare Cause of Secondary Pulmonary Arterial Hypertension

Abstract Unilateral absent pulmonary artery (UAPA) is a rare congenital disorder. Most of the patients will be diagnosed in the early childhood because of recurrent respiratory tract infections and hemoptysis, but adult presentation is not uncommon. We report a 47-year-old female who was earlier diagnosed as bronchiectasis with pulmonary artery hypertension but now presented with complaints of typical angina and dyspnea on exertion. During right heart catheterization we failed to enter right pulmonary artery (RPA), and conventional coronary angiogram showed a large left atrial branch of left circumflex giving collateral blood supply to the right lung. Computed tomography angiogram showed absent RPA. We report this case not only because the patient was misdiagnosed but also because of rarity of coronary collaterals in UAPA patients and unusual bilateral bronchiectasis. According to reported literature, ours is the 28th case of this nature.

324 Unilateral pulmonary artery agenesis with controlateral embolism: a double vascular trouble

Abstract Methods and results A 60-year-old male patient underwent coronary angiography (CA) for a non-ST segment elevation myocardial infarction (NSTEMI). CA revealed significant multivessel disease. Both internal mammary arteries (AMI) were patent, with right IMA markedly larger than the left IMA. The exam revealed also an abnormal branch arising from the proximal right coronary artery extending backwards, likely to the right lung. Pre-operative chest radiograph demonstrated asymmetry of the two hemithoraces with slight elevation of the right hemidiaphragm, small ipsilateral lung, and mediastinal shift towards the right. The patient underwent urgent CABG surgery. Myocardial revascularization was successfully performed using both AMI and one saphenous vein segment. The postoperative course was complicated by respiratory failure requiring prolonged mechanical ventilation. A chest computed tomography angiography was performed, revealing complete absence of the right pulmonary artery and a left lower lobe segmental pulmonary embolism. Furthermore, blood in the hypoplastic right lung was supplied by multiple collaterals arising from RCA and right IMA. Intravenous heparin was started with clinical improvement. Two weeks later, a lung scintigraphy was performed, ruling out perfusion defects. The patient was discharged home on oral anticoagulation with warfarin. Conclusions Unilateral pulmonary artery agenesis (UPAA) is an uncommon congenital anomaly of the great vessels. Despite the absence of the pulmonary artery, blood supply of ipsilateral lung is provided by systemic collaterals originating from bronchial, intercostal, internal mammary, and sub-diaphragmatic arteries. More rarely, these collaterals may arise from the coronary arteries with different implications, ranging from asymptomatic condition to myocardial ischaemia and infarction. In our case, the condition was previously asymptomatic. The occurrence of pulmonary embolism contributed to worsen the ventilation–perfusion mismatch, explaining the respiratory failure during the postoperative period.