A 53-year-old man with Eisenmenger syndrome (ES), caused by a large secundum atrial septal defect (ASD) and severe pulmonary arterial hypertension (PAH), showed a marked hemodynamic improvement following an escalation of PAH-specific therapy. Surgical ASD closure using a fenestrated patch was successfully performed after a significant reduction in both the patient's pulmonary pressure and vascular resistance. This case highlights the potential utility of the "treat-and-repair" strategy in patients with ES and severe PAH, who exhibit a favorable response to medical therapy, resulting in both a symptomatic and hemodynamic improvement.

Objectives: To evaluate the clinical spectrum of ventricular septal defect (VSD) in adolescents and adults. Methodology: A descriptive, observational study was conducted at the Pediatric Cardiology Department of Faisalabad Institute of Cardiology (FIC), Pakistan, from February to July 2023. Patients aged ≥10 years with unoperated VSD were enrolled, and the diagnosis was confirmed via transthoracic echocardiography. The clinical presentation, type of VSD, associated cardiac conditions, and complications were analyzed. Results: A total of 128 patients were included, with 60.9% aged 10–18 years and 39.1% aged over 18 years. Males accounted for 55.5% of the cohort. Most patients (89.1%) presented at the outpatient department. Perimembranous VSD was the most common type (58.6%), followed by sub-arterial (23.4%), inlet (7.8%), and muscular (7.8%) defects. Pulmonary hypertension (21.9%), aortic valve prolapse (12.5%), and mitral regurgitation (10.2%) were the most frequent associations. Eisenmenger syndrome (31.3%) was the most common complication. Older patients were significantly more likely to have pulmonary arterial hypertension (PAH) (p = 0.0006) and Eisenmenger syndrome (p = 0.0002). A significant association was found between VSD type and aortic valve prolapse (p < 0.0001). A p-value < 0.05 was considered statistically significant. Conclusion: Perimembranous VSD is the most prevalent defect in adolescents and adults. Many patients experience worsening functional status, with Eisenmenger syndrome emerging as a major complication. Early screening and timely intervention are crucial in preventing disease progression. Serial echocardiography should be incorporated into follow-up care for all unrepaired VSD patients. Establishing specialized adult congenital heart disease (ACHD) clinics in tertiary hospitals is essential for long-term management and improving patient outcomes.

Abstract Background Management of testicular germ cell tumors in patients with complex comorbidities remains challenging. We present a case of stage IIB seminoma in a patient with Down syndrome (DS) and Eisenmenger syndrome (ES). Case Presentation A 37-year-old man with DS and ES underwent radical orchiectomy for a testicular mass, confirming seminoma (pT2N2M0R0S0). Following disease progression during surveillance, external beam radiation therapy (36 Gy in 18 fractions) was administered using a dog-leg field technique, as both chemotherapy and surgery were contraindicated. Treatment was well-tolerated with only mild nausea. Follow-up imaging showed near-complete response at 4 months, with stable disease at 12-month follow-up. Conclusion This case demonstrates radiation therapy as an effective, well-tolerated treatment for stage IIB seminoma. Despite theoretical concerns regarding radiosensitivity in DS and the hemodynamic risks of thoracic irradiation in ES, standard para-aortic/iliac radiation was delivered safely, achieving disease control without unexpected toxicity.

The patient was a woman in her 60s with a history of congenital patent ductus arteriosus complicated by severe pulmonary hypertension and Eisenmenger syndrome. She had previously undergone surgery for right-sided breast cancer 11 years earlier. Nine years after surgery, she was diagnosed with bilateral breast cancer. At that time, mediastinal lymph node metastasis was suspected, and the disease was considered unresectable, leading to the initiation of pharmacological therapy. Although treatment was started, tumor progression was observed, prompting a shift toward surgery. Due to the presence of severe pulmonary hypertension, general anesthesia was deemed extremely high risk. Therefore, a surgical plan was made to perform bilateral mastectomy under local anesthesia with the aid of nerve blocks. Pectoral nerve blocks(Pecs blocks)combined with local anesthesia were used, resulting in effective pain control. No intraoperative or postoperative complications occurred. This case suggests that surgery using nerve blocks and local anesthesia may be a valuable option for patients with severe underlying and comorbid conditions in whom general anesthesia poses significant risk.

We report a case of successful treatment for a severe acute pulmonary hypertension crisis in a patient with Eisenmenger syndrome (ES) associated with patent ductus arteriosus following trauma-related spine surgery, utilizing both veno-arterial (V-A) and veno-venous (V-V) ECMO. The patient's clinical course was complicated by right ventricular failure amid a pulmonary hypertensive crisis, necessitating V-A ECMO support. Persistent hypoxia, despite improved right heart function, warranted transition to V-V ECMO. This case demonstrates that peripheral V-A ECMO can be safely employed to decompress the right heart in the presence of a significant PDA shunt. Furthermore, weaning from ECMO in these patients requires a much higher hematocrit in order to compensate for the chronic hypoxia.

Introduction: As survival in congenital heart disease (CHD) improves, identifying modifiable cardiovascular risk factors like dyslipidemia becomes increasingly important, though its features in adult CHD remain understudied. Methods: A retrospective study of 521 CHD patients (mean age 34 ± 14 years, 58% male) and 1782 matched controls (mean age 33 ± 11 years, 55% male) was conducted. Lipid profiles were assessed cross-sectionally at a single time point, and arterial thrombosis events were recorded over a mean follow-up of 5.8 years. Results: CHD patients had significantly lower total cholesterol (164.5 vs. 180.6 mg/dL), LDL (94.9 vs. 107.0 mg/dL), HDL (49.7 vs. 53.1 mg/dL), and triglycerides (97.0 vs. 102.4 mg/dL) compared to controls (all p < 0.05). Low HDL cholesterol (<40 mg/dL) was observed in 20% of CHD patients, nearly double the prevalence in the control group (11.6%; p < 0.001). This abnormality increased with CHD complexity: 15.2% in simple, 22.6% in moderate, and 28.9% in complex lesions. The proportion of patients with HDL < 40 mg/dL was higher in those with ventricular hypoplasia and Eisenmenger syndrome (p = 0.027). These groups also exhibited significantly higher NT-pro BNP levels, with a trend toward elevated C-reactive protein (CRP). Arterial thrombosis occurred in 4.0% of CHD patients versus 0.5% of controls (p < 0.001), with no significant differences between CHD subtypes. While overall lipid parameters did not differ significantly, the combination of low HDL and high LDL levels (mixed LDL + HDL pattern) was more common among patients with thrombosis (p = 0.005), although this association lost significance in binary logistic regression analysis. Conclusions: CHD patients exhibit a distinct lipid profile marked by lower HDL levels, particularly in complex cases and high-risk subtypes. Although the mixed low HDL/high LDL pattern was linked to thrombosis, this association was not maintained in multivariable analysis.

BackgroundEisenmenger syndrome (ES) is a severe and fatal complication of uncorrected congenital heart disease characterised by the development of pulmonary arterial hypertension (PAH) due to chronic left-to-right shunting. Despite the increasing use of PAH-targeted therapies, evidence of their long-term effects on the survival of patients with ES remains limited.MethodsWe reviewed 101 consecutive adult patients with ES (69% female; mean age, 34±18 years) who were referred to our centre between December 1972 and December 2023. We investigated the clinical, haemodynamic and treatment data from medical records. The primary outcome measure was transplantation-free survival. The effect of PAH-targeted therapy on transplantation-free survival was analysed using a time-dependent Cox proportional hazards model.ResultsAmong the 101 patients, 57 (56%) received PAH-targeted therapy (PAH therapy group), while 44 (44%) did not (treatment-naïve group). In the PAH therapy group, 75% received combination therapy (25%, monotherapy; 56%, dual-combination therapy; and 19%, triple-combination therapy). The median follow-up time since diagnosis of ES was 8.8 (IQR: 4.1–22.3) years. The median follow-up transplantation-free survival rates were significantly higher in the PAH therapy group than in the treatment-naïve group (100%, 100% and 89% vs 95%, 66% and 53% at 1, 5 and 10 years, respectively; p<0.001). In the multivariate analysis adjusting for age, sex, WHO functional class (III/IV vs I/II) and PAH-targeted therapy, PAH-targeted therapy was independently associated with improved transplantation-free survival (adjusted HR=0.275, 95% CI 0.132 to 0.572).ConclusionsThe long-term survival of patients with ES who received PAH-targeted therapy was better than that of those who did not. These results support the benefits of PAH-targeted therapies in this population while underscoring the need for large-scale studies to better define optimal treatment strategies.

Introduction: Eisenmenger syndrome (ES) in pregnancy is a catastrophic condition associated with maternal mortality rates of 30-50%. The profound physiological changes of pregnancy, particularly the decrease in systemic vascular resistance (SVR), exacerbate right-to-left (R-L) shunting, leading to severe hypoxemia and right ventricular failure. Anesthetic management is perilous, as both general and neuraxial anesthesia can precipitate hemodynamic collapse. Case presentation: We present the case of a 25-year-old G2P101Ab000 parturient at 32-34 weeks of gestation with ES secondary to a large secundum atrial septal defect and severe pulmonary hypertension. She presented for an urgent Cesarean section due to labor. A meticulous anesthetic plan was executed, centered on a low-dose Combined Spinal-Epidural (CSE) technique. This involved an intrathecal injection of 7.5 mg hyperbaric bupivacaine with 50 mcg fentanyl, followed by incremental epidural titration of 0.2% ropivacaine. Hemodynamic stability was proactively managed with inline infusions of phenylephrine and milrinone. The procedure was successful, maintaining stable maternal hemodynamics, SVR, and oxygen saturation. A healthy infant was delivered with APGAR scores of 7 and 8. The patient had an uncomplicated postoperative recovery. Conclusion: This case demonstrates that a carefully titrated, low-dose CSE technique, combined with invasive monitoring and proactive pharmacological support, can be a safe and effective strategy for Cesarean section in ES patients. This approach successfully navigates the hemodynamic dilemma by providing excellent analgesia while preventing a clinically significant drop in SVR.

Background: Atrial Septal Defect (ASD) is a common congenital heart anomaly characterized by a persistent interatrial communication, leading to a left-to-right shunt. If left untreated, significant defects can cause right heart volume overload, pulmonary hypertension, atrial arrhythmias, and heart failure. Aim: This article aims to provide a comprehensive overview of the clinical management of ASD, emphasizing a multidisciplinary approach that integrates insights from nursing, radiology, and laboratory sciences to optimize patient outcomes from diagnosis through long-term follow-up. Methods: The review synthesizes current knowledge on ASD, covering its etiology, epidemiology, pathophysiology, and clinical presentation. It details the diagnostic evaluation, primarily via transthoracic and transesophageal echocardiography, with advanced cross-sectional imaging (CT/MRI) for complex cases. Management strategies, including conservative surveillance, percutaneous device closure for suitable secundum defects, and surgical repair for complex anatomies, are examined. Results: Small ASDs may close spontaneously, but hemodynamically significant defects (Qp/Qs &gt;1.5) require intervention to prevent complications. Percutaneous closure is effective for secundum defects with adequate rims, while surgical repair is necessary for primum, sinus venosus, and coronary sinus defects. Timely closure typically leads to reverse right heart remodeling and excellent long-term prognosis, whereas delayed treatment risks irreversible pulmonary vascular disease (Eisenmenger syndrome). Conclusion: Effective ASD management relies on accurate anatomic and hemodynamic assessment to guide intervention. A coordinated, multidisciplinary team is essential for timely diagnosis, appropriate selection of closure method, and lifelong patient surveillance to prevent complications and ensure optimal quality of life.

Specific risk factors for heart-lung transplantation.

Congenital heart defects encompass a wide spectrum of varying anatomies and physiologies. Residuals after surgical or interventional treatment during childhood significantly shape the clinical course, as do multiple organ involvement and the development of degenerative diseases. As adults, many patients with congenital heart defects are no longer treated within specialized care structures, which is associated with a worsening prognosis and an increased frequency of emergency situations. Without appropriate expertise, emergencies in patients with complex congenital heart defects are challenging to assess. Arrhythmias and heart failure are the most common emergency scenarios in adults with congenital heart defects. Atrial tachycardias, particularly in the context of a Fontan circulation or a systemic right ventricle, should be treated promptly. Since the overall risk of sudden cardiac death is increased, syncope must always be thoroughly investigated. Depending on the specific anatomy and physiology, different therapeutic strategies must be employed for heart failure. In Eisenmenger syndrome, chronic cyanosis with reactive polycythemia and multiple organ involvement is the primary concern. Pregnancy and the peripartum period may precipitate decompensation of previously compensated conditions in patients with congenital heart disease. The ABCDE approach (Airway, Breathing, Circulation, Disability, Exposure) can generally be followed. However, without knowledge of the patient's medical history, treatment errors with potentially severe consequences can easily occur. By adding the letter F to the ABCDE scheme, the importance of the patient's Former medical history and additional information (Further information) in this vulnerable patient group is emphasized. A multidisciplinary approach and close coordination with a specialized adult congenital heart disease (ACHD) center are critical for improving the prognosis in acute situations.

Background There is a paucity of newer studies focusing on survival patterns in the era of targeted pulmonary vasodilators in Eisenmenger syndrome (ES). We aimed to study the survival pattern, mortality predictors and outcome of targeted pulmonary vasodilator therapy in patients with ES. Methods This was a single-centre retrospective observational study of patients treated between January 2000 and January 2020. Consecutive patients with clinical and echo- cardiographic and/or cardiac catheterization diagnoses of ES were included in the study. Shunt types were classified into pre-tricuspid shunts, post-tricuspid shunts and complex lesions. Clinical outcomes and treatment data of all patients were assessed. Results Of 206 patients, 139 were female with a mean (SD) age of 23.4 (14.3) years. Pre-tricuspid shunts were the most common shunt type, with atrial septal defect being the most common anatomical defect. During a mean (SD) follow-up of 7.1 (6.2) years, a significant decline in mean resting systemic saturation and WHO functional class on follow-up was observed. Atrial arrhythmias and right ventricular dysfunction were the most common in pretricuspid shunt lesions. ES secondary to complex defects had the worst prognosis with early attrition. Survival patterns were equally poor in complex defects and pre-tricuspid defects after the onset of Eisenmenger physiology. Targeted pulmonary vasodilator therapy was associated with better survival, irrespective of monotherapy or dual therapy. Congestive heart failure was the most common cause of death. Survival at 5 and 10 years in the disease modifier treatment group was 84% and 69% while in the treatment naïve group was 66% and 44%. Resting systemic saturation <80% on follow-up, the presence of atrial arrhythmias and the absence of disease-targeted therapy were independently associated with mortality. Conclusion The long-term survival of patients with ES remains poor in the era of targeted pulmonary vasodilator therapy. ES resulting from complex cyanotic congenital heart diseases has the worst survival outcomes. Although pretricuspid shunt ES patients survived longer, they tended to decompensate at lower mean pulmonary artery pressure than all other subgroups with ES. Targeted pulmonary vasodilator therapy may be associated with improved functional class and survival benefits in ES.

ABSTRACTPatients with Eisenmenger syndrome (ES) are at increased risk of thrombotic events, especially in pulmonary vessels. However, both the prevalence and risk factors of pulmonary arterial thrombosis (PAT) have been varied greatly. In this study we sought to examine the prevalence of PAT in adult patients with ES and to identify risk factors. A cross‐section descriptive study examining 57 adult patients with ES. All patients underwent computed tomography pulmonary angiography (CTPA) and echocardiography. Echocardiographic parameters and laboratory data were analysed from 57 patients. PAT prevalence was 22.8% (13/57), with all thrombi localized to dilated proximal pulmonary arteries (main pulmonary artery diameter: 52.6 ± 6.2 vs. 40.1 ± 6.9 mm in non‐thrombosis group, p < 0.05). Thrombotic patients exhibited higher D‐dimer (0.7 [IQR: 0.5–1.9] vs. 0.3 [IQR: 0.2–0.6] mg/L, p < 0.05) and NT‐proBNP levels (2561.0 [1389.0–5904.3] vs. 695.4 [180.6–2452.7] pg/mL, p < 0.05), alongside reduced right ventricular fractional area change (RV FAC: 23.9 ± 5.5% vs. 32.4 ± 10.0%, p < 0.05). No differences were observed in age, oxygen saturation, hemoglobin, coagulation profiles, or right heart catheterization parameters (mPAP, PVR, mRAP). PAT affects nearly one‐quarter of ES patients, routine CTPA screening should be considered even in asymptomatic cases. The lack of association with conventional risk factors underscores the need for thrombosis risk stratification integrating anatomical and biomarker criteria.

ABSTRACTAtrial septal defect (ASD) is a prevalent congenital heart abnormality that can be effectively repaired through either surgical or interventional procedures. We decide to describe a 50‐year‐old male with a history of dyspnea, cyanosis, and hypoxemia. A remarkable past medical history is that he underwent surgical correction of an ASD at the age of 6. Because of an initial misdiagnosis of Eisenmenger syndrome, he recently received palliative therapy, including therapeutic phlebotomy. Subsequent coronary computed tomography angiography (CTA) revealed systemic‐to‐pulmonary collaterals (SPCs) and an iatrogenic diversion of the inferior vena cava (IVC) to the left atrium (LA). A contrast study in transesophageal echocardiography confirmed the IVC's aberrant pathway to the LA. The diagnostic pathway and rationale behind these findings underscore the significance of multimodality imaging and reevaluation of the initial diagnostic impression.

Type I Truncus Arteriosus is a rare congenital heart defect characterized by a single arterial trunk arising from the heart, which causes mixing of oxygen-rich and oxygen-poor blood. Prompt surgical correction is essential to separate the pulmonary and systemic circulations, significantly enhancing patient outcomes. In this report, we present the case of a 22-year-old man who exhibited digital clubbing and cyanosis, along with a history of chronic exertional dyspnoea. He was diagnosed with Type I Truncus Arteriosus. This case offers important insights for healthcare professionals managing similar conditions, emphasizing the critical need for early diagnosis and timely intervention in patients with congenital heart disease (CHD) at risk of progressing to Eisenmenger syndrome. Clinicians should be well-informed and vigilant in identifying the signs and symptoms of pulmonary hypertension, as well as the possible complications that can develop as the disease advances.

ABSTRACTTo analyze the clinical characteristics and potential pregnancy outcomes of deceased pregnant women with pulmonary hypertension, we conducted a retrospective analysis of clinical data from 54 cases of pregnant women with pulmonary hypertension at The Third Affiliated Hospital of Guangzhou Medical University from May 2009 to February 2022. The results demonstrated that (1) Among 54 deceased pregnant patients with pulmonary hypertension (PH), 44 patients belonged to type 1, and 3, 2, and 5 patients belonged to type 2, type 4, and type 5, respectively. In type 1, 33 cases were secondary to congenital heart disease, with ventricular septal defect being common. (2) All 54 patients were diagnosed with PAH during pregnancy or postpartum. NYHA cardiac function grade II (10 cases), Ⅲ (18 cases), Ⅳ (26 cases), heart disease pregnancy risk levels were all Ⅳ. (3) Of the 54 patients, 7 patients had no data on the time of death, and most of them (37 patients) died within 1 week postpartum. (4) These 9 patients with mild PAH death during pregnancy had lower ejection fraction, higher rates of postpartum hemorrhage, left heart failure, systemic failure, arrhythmia, gestational diabetes, and infection than those with moderate or severe PAH death during pregnancy. As a consequence, deaths in pregnancies complicated with PH were mostly caused by PAH crisis, total heart failure, multiple organ failure, Eisenmenger syndrome and maternal perinatal mortality was high. The primary causes of mortality in pregnant women with mild PH include heart failure, respiratory failure, and arrhythmia etc. Patients experiencing complications such as postpartum hemorrhage, left ventricular dysfunction, multiorgan failure, arrhythmia, gestational diabetes mellitus, and infection may have a poorer prognosis.

The image represents the post-mortem heart of a 28-year-old female patient, diagnosed in childhood with complete common atrioventricular canal defect. At time of diagnosis, the family refused surgery, as did the patient during her adulthood. Despite being advised against pregnancy, she became pregnant. On presentation to hospital, she was cyanotic, with clubbed fingers, and hemodynamically unstable, in sinus rhythm, with Eisenmenger syndrome and respiratory failure partially responsive to oxygen. During pregnancy, owing to systemic vasodilatation, the right-to-left shunt is increased, with more severe cyanosis and low cardiac output. Echocardiography revealed the complete common atrioventricular canal defect, with a single atrioventricular valve with severe regurgitation, right ventricular hypertrophy, pulmonary artery dilatation, severe pulmonary hypertension and a hypoplastic left ventricle. The gestational age at delivery was 38 weeks. She gave birth to a healthy boy, with an Apgar score of 10. The vaginal delivery was chosen by an interdisciplinary team. The cesarean delivery and the anesthesia were considered too risky compared to vaginal delivery. Three days later, the patient died. The autopsy revealed hepatomegaly, a greatly hypertrophied right ventricle with a purplish clot ascending the dilated pulmonary arteries and a hypoplastic left ventricle with a narrowed chamber. A single valve was observed between the atria and ventricles, making all four heart chambers communicate, also insufficiently developed interventricular septum and its congenital absence in the cranial third. These morphological changes indicate the complete common atrioventricular canal defect, with right ventricular dominance, which is a rare and impressive malformation that requires mandatory treatment in early childhood in order for the condition to be solved.

Lung transplantation (LTx) for patients with pulmonary arterial hypertension (PAH) is associated with high postoperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) supports patients' haemodynamics during and after LTx. However, the optimal ECMO strategy, especially for patients with PAH, is debated. Here, we report our unique strategy for patients with PAH, using postoperative central veno-arterial (VA)-ECMO combined with delayed chest closure. This was a retrospective single-centre study of consecutive bilateral lung transplantations for adult patients with PAH performed between 2021 and 2024. Patients' characteristics, perioperative ECMO strategy, and postoperative outcomes were reviewed. During the study period, 20 PAH patients (idiopathic or hereditary PAH [n = 17], PAH secondary to collagen disease [n = 1], pulmonary veno-occlusive disease [n = 1], and Eisenmenger syndrome [n = 1]) underwent cadaveric LTx. Intraoperative support comprised either central VA-ECMO (n = 17) or cardiopulmonary bypass (n = 3). In 17 patients, central VA-ECMO was maintained postoperatively with temporary skin closure. The reason for postoperative central VA-ECMO was anticipated post-LTx heart failure due to PAH and suboptimal cardiac function. The median duration of ECMO support was 4 days (interquartile range: 2-4). There were 9 (45.0%) haemothorax evacuations while patients were on postoperative central VA-ECMO. No patients experienced haemodynamic collapse after LTx. All patients survived during the observation period, resulting in a 100% survival rate at both 90 days and 1 year (95% confidence interval: 83.2%-100%). Extended postoperative central VA-ECMO and delayed chest closure were feasible for patients with PAH who underwent LTx. Meticulous haemostasis is mandatory, given the high chance of haemothorax evacuation.

Introduction: Atrioventricular canal defect (AVCD) is a congenital heart malformation characterized by a variable degree of atrial and ventricular septal defect [1] . Anatomically, there are partial, complete and intermediate forms [2] . This first Malagasy study was designed to describe its epidemio -clinical aspects in children with AVCD and to determine the factors influencing the prognosis but also to serve as a database of AVCD in Madagascar. Method: This was a retrospective, descriptive and analytical study over a period of 20 years on 98 patients with AVCD seen at the Soavinandriana Hospital Center (CENHOSOA) Antananarivo. Results: We found that AVCD represented 4.70% of congenital heart diseases observed with a mean age of discovery of 29.24 months and a male predominance (sex ratio of 1.61). The complete form represented 84.54% of cases. Trisomy-21 was found in 46.39% of patients. Atrial septal defect (ASD) (12.37%) and ventricular septal defect (VSD) (8.25%) were the main associated cardiac malformations followed by transposition of the great vessels (8.25%). Signs of heart failure were present in 27.83% of cases and 34.02% had pulmonary hypertension (PH) of which 11.34% had Eisenmenger syndrome. In this study, age of discovery more than 12 months was associated with the onset of PH and Eisenmenger syndrome (p = 0.03 and 0.01). Non-trisomy patients tend to present with heart failure and PH (p = 0.02 and 0.01). Conclusion: Cardiovascular exploration is required in all infants with Down syndrome and those who present signs of CAV. Delayed diagnosis remains the main factor in poor prognosis of CAV in Madagascar.

BackgroundPregnancy in patients with significant pulmonary hypertension (PH) is a contraindication due to high maternal and fetal mortality. However, recent data shows that an increasing number of women with PH are successfully carrying pregnancies to term, likely because of advancements in multidisciplinary care and treatment. This systematic review and meta-analysis aims to evaluate recent outcomes and identify risk factors associated with morbidity and mortality in this high-risk group.MethodsAn unrestricted search was conducted across MEDLINE, EMBASE, and Cochrane databases for studies on outcomes in pregnant patients with PH from inception until May 30, 2024. For one-group analyses, event rates and 95% confidence interval (CI) were calculated using a random-effects model. Meta-regression was performed with Comprehensive Meta-Analysis version 4, employing covariates in logistic regression models. For two-group comparisons, odd ratios (ORs) and 95% CIs were calculated using Review Manager version 5.4 utilizing a random-effects model.ResultsFifty-one studies involving 4,583 pregnant patients with PH were included. The maternal mortality rate was 7.6% (95% CI: 5.8–9.9%). Most deaths (93%) occurred in the postpartum period. Severe PH was associated with a significantly higher rate of maternal mortality compared to mild/moderate PH with OR 5.57 (95% CI: 3.12–9.94). Higher systolic pulmonary artery pressure (sPAP) was associated with a higher mortality rate with a regression coefficient of 0.96. Similarly, the presence of Eisenmenger syndrome (ES) was linked to a higher mortality rate with a regression coefficient of 1. Severe PH was also associated with higher rates of abortion (OR =3.64, 95% CI: 2.61–5.09), prematurity (OR =2.52, 95% CI: 1.66–3.81), and small gestational age (OR =3.96, 95% CI: 2.96–5.29) compared with mild/moderate PH.ConclusionsAlthough the outcomes of pregnant patients with PH continue to improve, severe PH continues to be associated with a high mortality rate, abortion rate, prematurity, and small gestational age. Our findings support the existing consensus against pregnancy in severe PH and emphasize the need for thorough, individualized discussions about risks for patients with mild/moderate PH.