Irreversible Pulmonary Hypertension Research Articles

Total artificial heart: past, present, and future

Every year, approximately 5 out of 1000 patients receive a diagnosis of advanced heart failure, with a prevalence of 1-2% in the adult population. This figure is likely underestimated, considering undiagnosed cases. Despite significant progress in medical therapy for heart failure, mortality rates persist around 20% within the first year, reaching 50-60% at 5 years from the initial diagnosis. For patients with severe end-stage heart failure, the 1-year mortality rate can reach up to 70%. Heart transplantation remains the preferred treatment for terminal stages of the disease; however, the significant challenge lies in the mismatch between available donors and recipients. Given this dilemma, both short-term solutions including extracorporeal membrane oxygenation and long-term options such as left ventricular assist devices have gained prominence. These mechanical circulatory support systems become crucial for patients in critical conditions, temporarily ineligible for heart transplantation, such as those with severe irreversible pulmonary hypertension or acute organ failure. Despite these advancements, a growing number of patients on the waiting list develops severe biventricular dysfunction, precluding the use of a left ventricular assist device as a bridge to transplant. In such cases, a total artificial heart emerges as a viable therapeutic option.

Echocardiographic assessment of right ventricular function status in children with congenital heart disease with irreversible and reversible pulmonary hypertension compared to normal children

Background: Right ventricular (RV) dysfunction is prevalent in children with congenital heart disease (CHD), particularly in association with pulmonary hypertension (PH). Transthoracic echocardiography (TTE) is crucial for detecting, assessing severity, and monitoring CHD patients with PH. The current study aimed to compare RV function assessed by TTE between CHD patients with PH and healthy children. Methods: The case group comprised CHD patients diagnosed with PH via TTE and confirmed by cardiac catheterization. The control group included children with normal echocardiograms. A total of 40 patients were enrolled based on predefined criteria. Results: The study included 20 cases and 20 controls. Echocardiographic parameters revealed significant differences between the case and control groups, with increased RV dimensions in the case group, except for RV subcostal wall thickness. RV systolic function parameters, including tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (S'), were significantly lower in the case group. RV diastolic function parameters, including E/A ratio, E/e', and deceleration time (DT), were also significantly different between the groups. The RV global function parameter, tissue Doppler imaging-derived myocardial performance index (TD MPI), was significantly higher in the case group. Conclusions: Echocardiographic evaluation of RV function, including TAPSE, S', TD MPI, E/A ratio, E/e', and DT, in CHD children with PH correlates with PH severity. This assessment can aid in early detection of PH severity before irreversible changes occur and serve as an alternative to right heart catheterization.

Influence of cardiac diagnosis on outcomes of pediatric heart-lung transplantation.

Pediatric Heart-lung transplant (HLTX) is performed for endstage congenital heart disease (CHD) with irreversible pulmonary hypertension or non-congenital heart disease (NCHD) with end-stage heart and lung disease. CHD could influence the outcomes of HTLX due to increased complexity of the operation as compared to NCHD. In this study we evaluated the influence of cardiac diagnosis on outcomes of pediatric HTLX. The UNOS database (1987-2022) was queried for primary HTLX in patients <18years. The data were extracted for demographics, pretransplant characteristics, post-transplant outcomes, and analyzed for the impact of cardiac diagnosis on post-transplant outcomes. Standard statistical tests were used. Survival was compared using the Kaplan-Meier method. Ninety of the 213 patients who underwent HLTX had CHD. There were no demographic differences. Heart listing status was similar but with a higher LAS score for NCHD. NCHD had higher pre-operative life support use (mechanical ventilation, inotropes or dialysis) but the use of ECMO as a bridge to transplantation was similar. Wait-list times were longer for CHD. The ischemic times were similar. Post-transplant dialysis, stroke, prolonged mechanical ventilation, and rejection were similar. Survival at 30-days, 1-year, and long-term survival at 17years was similar. Non-survivors at 30-days post-transplant were on life support, used ECMO as a bridge, had lower wait-list times, longer ischemic times and had strokes. Non-survivors at 1-year had similar factors in addition to a higher dialysis use. Cardiac diagnosis had no impact on outcomes after Pediatric HLTX. Patients on life support or ECMO before transplantation were transplanted faster but with lower survival.

Autologous Reconstruction of Anomalous Origin of a Right Pulmonary Artery From the Aorta.

Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.

Remimazolam in perioperative management of Eisenmenger syndrome: a case report.

Eisenmenger syndrome (ES) is characterized by severe and irreversible pulmonary hypertension stemming from an uncorrected intracardiac shunt of significant size. The imbalance between systemic and pulmonary artery pressures predisposes patients with ES to the risk of cardiac arrest. Remimazolam has caused less circulatory depression, which may be advantageous for ES. However, no studies reported the use of remimazolam in perioperative ES management. A 45-year-old female patient with ES derived from a ventricular septal defect was scheduled to undergo bilateral adnexectomy for an ovarian tumor. Her oxygen saturation was 80% with 3 L/min oxygen at rest, and her pulmonary and systemic flow ratio was 0.53. She underwent general anesthesia with remimazolam, and intraoperative hemodynamics was stable without hypotension or reduced oxygen saturation. Our successful management of ovarian tumor surgery in a patient with ES using remimazolam reveals its potential effectiveness in perioperative care.

Abstract 13091: Chronic Thromboembolic Pulmonary Hypertension Associated With Prolonged Use of Combined Oral Contraceptives in a 27-Year-Old Woman: A Successful Case of Pulmonary Endarterectomy

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with venous thromboembolism (VTE); however, in half of the cases, no prior deep vein thrombosis is ever found. The use of combined oral contraceptives (COCs) is a known risk factor for VTE. We present the case of a young woman with CTEPH in whom timely diagnosis and intervention prevented irreversible pulmonary hypertension (PH). Case Presentation: A 27-year-old woman with 10-year use of COCs presented with progressive dyspnea for 3 weeks. The ECG showed right ventricular systolic overload and right atrial enlargement. Laboratory tests showed elevated pro-BNP and D-dimer. TTE showed right atrium and right ventricle (RV) enlargement and elevated right ventricular systolic pressure (RVSP) of 83 mmHg. The ventilation/perfusion scintigraphy showed mismatched segmental perfusion defects in both lung fields with greater involvement of the left lung, and oral anticoagulation was started. Lower extremity venous ultrasound and hypercoagulability studies were normal. CTPA revealed filling defects in the left main pulmonary artery and bilateral segmental branches. After 3 months of anticoagulation, she persisted in NYHA class III, and right heart catheterization showed mean PAP of 38 mmHg, PCWP of 8 mmHg, and PVR of 9 WU, confirming CTEPH. Due to unavailability in Peru, she was referred to a US center for pulmonary endarterectomy (PEA). After a successful intervention, she improved clinically with a reduction in RVSP to 34 mmHg. This case highlights the importance of rapid intervention in CTEPH even in situations where optimal treatment is not readily available. Conclusion: CTEPH may be associated with the prolonged use of COCs in young women. Timely intervention can reverse PH and prevent RV remodeling. Unfortunately, Peru does not have a PEA center and not every case will undergo treatment. We call for the training of local surgeons to ensure fair and equitable access for the population.

Metabolomics in Pulmonary Hypertension-A Useful Tool to Provide Insights into the Dark Side of a Tricky Pathology.

Pulmonary hypertension (PH) is a multifaceted illness causing clinical manifestations like dyspnea, fatigue, and cyanosis. If left untreated, it often evolves into irreversible pulmonary arterial hypertension (PAH), leading to death. Metabolomics is a laboratory technique capable of providing insights into the metabolic pathways that are responsible for a number of physiologic or pathologic events through the analysis of a biological fluid (such as blood, urine, and sputum) using proton nuclear magnetic resonance spectroscopy or mass spectrometry. A systematic review was finalized according to the PRISMA scheme, with the goal of providing an overview of the research papers released up to now on the application of metabolomics to PH/PAH. So, eighty-five papers were identified, of which twenty-four concerning PH, and sixty-one regarding PAH. We found that, from a metabolic standpoint, the hallmarks of the disease onset and progression are an increase in glycolysis and impaired mitochondrial respiration. Oxidation is exacerbated as well. Specific metabolic fingerprints allow the characterization of some of the specific PH and PAH subtypes. Overall, metabolomics provides insights into the biological processes happening in the body of a subject suffering from PH/PAH. The disarranged metabolic pathways underpinning the disease may be the target of new therapeutic agents. Metabolomics will allow investigators to make a step forward towards personalized medicine.

Surgical Treatment of Congenital Heart Diseases Associated with Pulmonary Hypertension in Adults

The outcome of treatment of adult patients with congenital heart disease (CHD) and pulmonary hypertension (PH) depends on the adequacy of diagnosis with a thorough assessment of hemodynamics and the correctness of the chosen tactics. Enormous success has been achieved, various classifications and algorithms for managing children and adults with this nosology have been created and are widely used. Thanks to the creation and widespread introduction into clinical practice of various types of drugs, the results of surgical treatment of CHD with PH have improved significantly. A new cluster of patients has emerged who can undergo palliative surgery against the background of irreversible pulmonary hypertension, leading to a significant improvement in the quality and life expectancy. At the same time, to this day, it is far from always possible to evaluate each specific case with full confidence in the possibility of performing surgical treatment. Modern advances in CHD cardiac surgery have significantly expanded the indications for the correction of defects with high PH in adults. Operability (implying normalization of pulmonary artery pressure) and indications for palliative surgery (without regression of PH, but with an improvement in the quality and life expectancy of patients) are still the main debatable issues.

Pulmonary capillary hemangiomatosis: an unusual cause of primary pulmonary hypertension in pediatric age group

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. PCH’s main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations and later irreversible pulmonary hypertension and right-sid­ed heart failure. It is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography and pulmonary congestion triggered by pulmonary vasodilating therapy. The imaging features include diffuse centrilobular ground‑glass opacities with features of pulmonary hypertension. We present a case of PCH in a 4‑year‑old boy who was diagnosed with post-partum hemorrhage (PPH) in echocardiography and computed tomography of the thorax.

Late surgical ventricular septal defect closure in a low middle-income country setting: a case series

BackgroundVentricular septal defect (VSD) is the commonest type of congenital heart lesion accounting for up to 40% of congenital heart defects. Well timed VSD closures are reported to yield excellent long-term outcomes. Late surgical VSD closures, particularly from the developing countries, are infrequently reported.Case presentationWe report three cases of African children aged between 13 and 14 years who had late VSD presentations. They reported complaints of growth failure and recurrent respiratory infections since early infancy which necessitated frequent visits to primary health care facilities. They were found to have large ventricular septal defects by thoracic echocardiography. Diagnostic cardiac catheterization was done to all three patients to rule out irreversible pulmonary hypertension. After promising cardiac catheterization findings, they all underwent successful surgical VSD repair with good early outcomes.ConclusionVSD surgical closure is ideal in children below 2 years, however, it can be done in children who present at advanced age despite being considered high risk patients. All three of our patients who presented late had successful surgical VSD repairs with promising immediate outcome. The role of genetics in the protection against developing irreversible pulmonary vascular disease in these patients is a possible area for future studies.

A Patient with Right-to-Left Shunt Atrial Septal Defect Without Pulmonary Hypertension

We present a case of atrial septal defect (ASD) in a 27-year-old young woman who presented with a right-to-left shunt despite normal pulmonary artery pressure without anomalous pulmonary venous drainage and only mild tricuspid regurgitation (TR), but it was associated with right ventricular dysfunction. Considering the dysfunction of the right heart and significant dilatation of the tricuspid annulus, the surgeon still decided to repair ASD and perform tricuspid valvuloplasty. The surgery lasted approximately three hours, and the whole procedure was smooth and uneventful. Her postoperative recovery was uneventful and discharged in good condition. The occurrence of right-to-left (R-L) shunting in patients with ASD is uncommon and is usually an ominous sign, heralding irreversible pulmonary hypertension (PH). There is little information about similar shunts for patients without PH and tricuspid regurgitation (TR), and the majority of these patients have an anatomic anomaly that favors this type of shunt. ASD with R-L shunt without Eisenmenger physiology is rarely reported and may be underestimated due to difficulty in its diagnosis. Intraoperative transesophageal echocardiography (TEE) examination should not only focus on cardiac structure but also the evaluation of ventricular function. Right-to-left shunt is possible in ASD patients even with normal pulmonary arterial pressure.

Serial evaluation of bosentan monotherapy in a patient with Eisenmenger syndrome during an 11-year period: A case follow-up report

Eisenmenger syndrome is a rare disease primarily characterized by severe degrees of irreversible pulmonary arterial hypertension in the setting of congenital heart disease. In this report, we aim to report, on a yearly basis, the clinical, hemodynamic and echocardiographic parameters of a female patient with Eisenmenger syndrome who had been under bosentan treatment during an 11-year follow-up.

Challenges in device closure of PDA in adults.

Objective: To describe the challenges and outcomes of per cutaneous closure of PDA in adolescents and adults. Study Design: Cross Sectional, Retrospective Analysis. Setting: Department of Pediatric Cardiology, CPE Institute of Cardiology, Multan. Period: 2017 to 2019. Material &amp; Methods: A total of 111 adolescent and adult patients were included in the study. Patients with irreversible severe pulmonary hypertension were not included. Sampling with consecutive nonprobability was done. Procedure was done according to standard protocols. Different devices were used in different patients according to morphology of defect. Devices included SHSMA and Lifetech duct occluders. In two patients VSD muscular devices were also used. Results: Mean age was 22 + 7.88 years. Out of these 111 subjects, 72 (63%) were female and 39 (37%) were male with a ratio of 1.84:1. Two patients were of moderate size (3-5mm) PDA, 26 (27%) were of moderately large (5-7mm) size and majority of patients had large size (&gt;7mm) PDA (55%). Majority of PDAs were type A (100, 90%), one was type B, 4 were type C and 6 were of type E according to Krichenko classification. No complications occurred except in one patient in which device was embolized. Foaming through the device was noted in 65 patients after 24 hours post procedure echocardiography. Conclusion: It was concluded that PDA device closure has its own challenges in adults but it is safe and effective as well.

Selection Criteria for Heart Transplantation in Patients with End-Stage Heart Failure

Today, heart transplantation remains the gold standard of treatment for patients with end-stage heart failure (HF). As the number of donors is limited and the risk of intervention is quite high, the key to achieving optimal outcomes and reducing the incidence of complications is the proper selection of patients who meet clearly defined criteria for inclusion in the waiting list for heart transplantation. This review presents the latest knowledge on indications and contraindications for inclusion recipients in the waiting list for heart transplantation, including the latest clinical guidelines of The International Society for Heart and Lung Transplantation (ISHLT) published in 2016.&#x0D; Thus, the absolute indications are dependence on intravenous inotropic and mechanical circulatory support; inoperable heart diseases or a history of volume reducing operations; refractory angina or life-threatening refractory arrhythmias despite maximal drug therapy and/or surgical correction; reduction of maximum oxygen consumption up to &lt;12-14 ml/kg/min when performing the maximum cardiopulmonary exercise test. While including patients in the waiting list for heart transplantation, it should be noted that the low left ventricular ejection fraction &lt;30%, a history of documented NYHA class III-IV HF, low maximum oxygen consumption as the only criterion of terminal HF and poor prognosis for annual survival in HF calculated on prognostic scales are not sufficient indications. Absolute contraindications include a systemic disease with a life expectancy of less than 2 years and irreversible pulmonary hypertension.&#x0D; The current procedure for the distribution of recipients by urgency status for heart transplantation is presented according to the updated recommendations of the United Network for Organ Sharing (UNOS) published in 2018 which consists of 6 degrees, compared with the 1999 edition which contained 3 degrees.

Aorto-pulmonary Window, a Rare Congenital Heart Disease: Case Report

Aortopulmonary window (APW) is an uncommon congenital cardiac malformation, accounting for 0.1% of all congenital cardiac diseases. It is a defect between the ascending aorta and the trunk of the pulmonary artery. Such abnormality may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. Clinical and hemodynamic presentation of this condition depends on the size of the defect and on the associated lesions. It is usually fatal in infancy or childhood if untreated with the development of irreversible pulmonary hypertension. Once the diagnosis is made, surgery must be performed quickly to avoid irreversible pulmonary vascular disease. We report the case of a 5-month-old infant with APW who was referred to our center by respiratory symptoms and heart murmurs.

Current Endovascular Approach in Adult Patients with Pulmonary Vein Stenosis: A State-of-the-Art Approach

Regarding to more invasive treatment of atrial arrhythmia (atrial fibrillation ablation) and pulmonary vein isolation (PVI), the rate of acquired pulmonary vein stenosis (PVS) is increasing and at present, PV ablation for AF has become the principal cause of PVS in adult patients. On the other way, by improvement in procedural techniques, equipment, and the experience of the operators, the incidence of PVS has been decreased. There is some controversy about the manner of follow-up of these patients and in most centers, just symptomatic patients are considered for imaging and treatment. Almost always, those with PV stenosis more than 70% or multiple PV involvement become symptomatic and if give them up without treatment, pulmonary symptoms and finally irreversible pulmonary hypertension will occurred. So, intensive pursue after the procedure is highly recommended. Whereas in pediatric patients with congenital or acquired PVS, the best treatment approach is surgery, in adult patients, the preferred type of treatment is the transcatheter intervention with high acute success rate. In this present review, we have scrutinized about the diagnostic modalities, the indications for intervention, the diverse treatment strategies, and principally clarify an accurate stepwise approach during transcatheter procedure.

Comparison of levosimendan, NO, and inhaled iloprost for pulmonary hypertension reversibility assessment in heart transplant candidates.

AimsAssessing reversibility of pulmonary vascular changes through vasoreactivity testing (VRT) optimizes end‐stage heart failure patient selection for heart transplant. All efforts should be made to unload the left ventricle and reduce pulmonary vascular resistance to effectively exclude irreversible pulmonary hypertension.Methods and resultsWe reviewed our centre's cardiac transplant registry database (2009–2017) for VRT and compared haemodynamic responses with 40 ppm inhaled NO (n = 14), 14–17 μg inhaled iloprost (n = 7), and 24 h 0.1 μg/kg/min intravenous levosimendan (n = 14). Response to levosimendan was assessed by repeat right heart catheterization within 72 h. Baseline clinical and haemodynamic features were similar between groups. VRT was well tolerated in all patients. All drugs effectively reduced pulmonary artery pressures and transpulmonary gradient while increasing cardiac index, although levosimendan had a greater impact on cardiac index increase (P = 0.036). Levosimendan was the only drug that reduced pulmonary artery wedge pressure (P = 0.004) and central venous pressures (P < 0.001) and increased both left and right ventricular stroke work indexes (P = 0.020 and P = 0.042, respectively) and cardiac power index (P < 0.001) compared with NO and iloprost. Right ventricular end‐diastolic pressures and central venous pressure were only decreased by levosimendan. The rate of positive responses (≥10 mmHg decrease or final mean pulmonary artery pressure ≤40 mmHg with increased/unaltered cardiac index) was lower with inhaled iloprost (14%) than with either levosimendan or NO (71% and 64%, respectively; P < 0.05).ConclusionsLevosimendan may be a safe and effective alternative for pulmonary hypertension reversibility assessment or a valuable pre‐test medical optimization tool in end‐stage heart failure patient assessment for heart transplantation offering extended haemodynamic benefits. Whether it increases the rate of positive responses or allows a better selection of candidates to heart transplantation remains to be established.

Validity Of Right Ventricular Strain In Prediction Of Successful Simple Cardiac Shunt Closure

Background: Pulmonary arterial hypertension in congenital heart disease is reversed by early shunt closure, but this potential is lost beyond a certain point of no return. this study was conducted to evaluate the results of simple cardiac shunt closure regarding right ventricular function using speckle tracking and reversibility of pulmonary hypertension, testing its ability to provide information with minimal risk,we detected right ventricular function in simple cardiac shunt patients before closure using longitudinal strain and to investigate its role in predicting reversibility of pulmonary hypertension after closure Methods: a prospective randomized comparative study,simple shunt patients from age 25-48 years presented to Zagazig University hospitals, cardiology department from September 2018 to July 2019 were divided into two groups Group I: ten patients having irreversible pulmonary hypertension. Group II: Twenty patients having reversible pulmonary hypertension. Results: There was statistically significant increase in Group II post closure regarding LVEF, RVFAC,TAPSE,SRV, RVLS (P< 0.05). While PASP showed significant decrease in Group II post closure (P < 0.05). On the other hand, RVLS was negatively correlated with the defect size (r = -0.65; p = <0.001), Qp/Qs (r = -0.53, p = <0.001), PASP (r = -0.8, p = <0.001). And significant positive correlation with SRV(r = 0.37; p = <0.001),RVFAC (r =0.30, p < 0.001),TAPSE(r =0.34, p < 0.001).ROC curve analysis revealed that RVLS value of ≥ -20 could predict reversibility of PASP (P< 0.001). Conclusion: The RVLS value of ≥-20 could predict reversibility of PASP (P< 0.001). RVLS correctly identify simple shunt patients with increased PASP.

Spontaneous closure of ventricular septal defects in newborns the first year of life

Abstract Background Ventricular septal defect (VSD) is one of the most common congenital heart defects. Some VSDs close spontaneously while persistence of the VSD may result in heart failure and, at long term, irreversible pulmonary hypertension. Consequently, identification of factors associated with spontaneous closure are important to ensure relevant clinical follow up of those at risk of a persistent VSD. Purpose We aimed to assess the rate of spontaneous closure in a population-based cohort of newborns with VSD and to identify significant predictors for spontaneous closure. Methods The study is part of a large-scale population-based study, focusing on cardiac structure and function, including newborns born from 1st April 2016 to 31st October 2018. Newborns with a VSDs at baseline were followed with echocardiography at 3, 6 and 12 months (+30 days) of age. Cox proportional hazard model was used to determine significant predictors of spontaneous closure Results Based on echocardiographies of 25.750 newborn, 850 newborns (3.3%) with a VSD were identified. The majority of newborns with VSDs were born at term (88.7%) and the mean age at inclusion was 11.7±8.1 days. VSDs were more frequent in females (57.1%, p&amp;lt;0.001). Of all VSDs, 787l (92.6%) were of a muscular type, 60 (7.0%) of perimembranous type and 3 (0.4%) were of subarterial type. Multiple VSDs were found in 98 (11.5%) cases and all were of a muscular type. Muscular VSDs were significantly smaller than perimembranous VSDs (1.9±0.7 mm vs. 3.7±1.8 mm, p&amp;lt;0.0001). During the follow up period, seven (0.8%) children underwent surgical repair: five (5 of 56) children with perimembranous VSDs and two (two of three) children with subarterial VSDs. Follow-up was complete in 89.4% of all children. After one year 82.6% (601 of 727) VSDs had closed spontaneously. The prevalence of VSD in one-year old children was thus 0.5%. The majority of VSD closed spontaneously during the first 6 months of life (57.6% (419/727)). Muscular VSDs showed significantly higher rate of spontaneous closure compared with perimembranous VSDs (86.9% (582 /670) vs. 46.2% (25/54), p=0.0001) during the first year of life. No subarterial VSDs closed spontaneously during follow-up. Muscular type (p=0.002), size (p&amp;lt;0.001) and presence of one VSD as opposed to multiple (p&amp;lt;0.0025) were associated to spontaneous closure. Sex did not significantly influence spontaneous closure rate (p=0.65). Conclusion In a population study including more than 25,000 newborns we found that the vast majority, i.e. almost 9/10 of all VSDs, closed spontaneously during the first year of life, ultimately resulting in a prevalence of VSD in one-year old children of 0.5%. The identified factors associated with spontaneous closure – anatomical location, size and presence of multiple VSDs are of use for future management of VSDs in newborns. Funding Acknowledgement Type of funding source: Other. Main funding source(s): The Danish Heart Foundation

Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review.

Congenital, isolated unilateral agenesis of pulmonary arteries (UAPA) has a bimodal clinical presentation. It is either diagnosed in early infancy or during adulthood. Early diagnosis and timely surgical correction at infancy prevent the development of irreversible pulmonary hypertension and enable prolonged survival. Though surgical intervention is a conclusive method of treatment, its outcome depends upon the reversibility of pulmonary hypertension and the adequacy of the pulmonary artery dimension. Adults with UAPA can be asymptomatic (diagnosed incidentally) or symptomatic. They may present clinically with myriads of symptoms like exertional dyspnea, recurrent lung infection, hemoptysis, and atypical chest pain. For cases where definitive surgical treatment is not feasible, interventional and medical management should be carried out as part of palliative therapy. A retrospective analysis of 22 cases of UAPA in adults published in the literature from the years 2017-2020 are presented here, along with our institutional case.