Eisenmenger Syndrome Research Articles

Successful Eisenmenger syndrome-targeted drug therapy in pregnant women: a case series and literature review.

To clarify the real-world outcomes in pregnant women with Eisenmenger syndrome (ES) in the new therapeutic era and provide a literature review. Retrospective case and literature review. Tertiary referral hospital (The Second Xiangya Hospital of Central South University). Thirteen women with ES delivered between 2011 and 2021. Respective study and literature reviews. Maternal and neonatal mortality and morbidity. 12/13 (92%) pregnant women were treated with targeted drugs. 9/13 (69%) of patients had heart failure, but no maternal deaths occurred. 12/13 (92%) of women chose caesarean delivery. One pregnant woman gave birth at 37+1 weeks, and the remaining 12 (92%) patients had preterm birth. 10/13 (77%) women gave birth to live infants, of which 9/10 (90%) were low birthweight infants with a mean birthweight of 1575 g. The infant mortality rate was 1/10 (10%). Cardiac functional class improved during pregnancy, probably due to therapy; 11/13 (85%) of the pregnant women were in cardiac functional level III/IV at admission and 12 (92%) were in cardiac functional class II/III at discharge. Our literature review identified 72 cases of pregnancy with ES from 11 studies, which were characterised by a low rate of targeted drug use (28%) and a high maternal mortality rate of 24% in the perinatal period. Our case series and literature review suggest that targeted drugs may be key to improving maternal mortality in ES.

Univentricular hearts not undergoing Fontan: the type of palliation matters

Introduction and objectivesThere is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

Eisenmenger´s syndrome with complete AV septal defect in a 55- year-old woman

Eisenmenger syndrome is characterized by an elevation of pulmonary pressure to systemic levels caused by an increase in pulmonary vascular resistance, with a severe shunt (right-left) or bidirectional shunt. This was a consequence of an uncorrected congenital defect. Its diagnosis is based on the clinical history, symptoms, echocardiography, and cardiac catheterization.

Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: Insights from a national multicenter prospective registry

The purpose of this registry was to provide insights into the characteristics, treatments and survival of patients with PAH-CHD in China. Patients diagnosed with PAH-CHD were enrolled in this national multicenter prospective registry. Baseline and follow-up data on clinical characteristics, PAH-targeted treatments and survival were collected. A total of 1060 PAH-CHD patients (mean age 31 years; 67.9% females) were included, with Eisenmenger syndrome (51.5%) being the most common form and atrial septal defects (37.3%) comprising the most frequent underlying defect. Approximately 33.0% of the patients were in World Health Organization functional class III to IV. The overall mean pulmonary arterial pressure and pulmonary vascular resistance were 67.1 (20.1) mm Hg and 1112.4 (705.9) dyn/s/cm5, respectively. PAH-targeted therapy was utilized in 826 patients (77.9%), and 203 patients (19.1%) received combination therapy. The estimated 1-, 3-, 5-, and 10-year survival rates of the overall cohort were 96.9%, 92.9%, 87.6% and 73.0%, respectively. Patients received combination therapy had significantly better survival than those with monotherapy (p=0.016). NT-proBNP >1400 pg/ml, SvO2 ≤ 65% and Borg dyspnea index ≥ 3 and PAH-targeted therapy were independent predictors of mortality. Hemoglobin > 160g/L was a unique predictor for mortality in Eisenmenger syndrome. Chinese PAH-CHD patients predominantly exhibit Eisenmenger syndrome and have significantly impaired exercise tolerance and right ventricular function at diagnosis, which are closely associated with long-term survival. PAH-targeted therapy including combination therapy showed a favorable effect on survival in PAH-CHD. The long-term survival of Chinese CHD-PAH patients remains to be improved.

Approach to hypoxemia in the emergency department in a clinical case of Eisenmenger Syndrome

There is a wide variety of pathologies that can cause hypoxia with different pathophysiological mechanisms, and therefore with different response to treatment with supplemental oxygen, it is essential for emergency care physicians to know the different pathologies causing hypoxia to be able to adequately and direct the treatment , in this paper we discuss the case of a patient with syncope and hypoxia who reaches the emergency department, which mechanism of hypoxia is a consequence of an interatrial short circuit, reaching the conclusion of Eisenmenger syndrome, which is a pulmonary vascular disease, with elevation of pulmonary resistance in patients with a history of heart disease with short circuit from left to right. This pathology is of vital importance that its identification is made in initial stages, with the aim of preventing evolution to the point where medical treatment is insufficient, and as in the case to mention, the only remaining option would be lung and heart transplantation.

Fruitful case of Eisenmenger syndrome with ventricular septal defect in a case of pregnancy: A case report

Fruitful case of Eisenmenger syndrome with ventricular septal defect in a case of pregnancy: A case report

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.

Pulmonary vascular resistance/systemic vascular resistance (PVR/SVR) ratio changes after sildenafil therapy in uncorrected congenital heart disease-associated pulmonary arterial hypertension

Pulmonary vascular resistance (PVR) to systemic vascular resistance (SVR) ratio is a prognostic predictor in congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after defect correction. Sildenafil, widely used as a PAH drug, can decrease PVR with minimal or without changes in SVR, resulting in decreased PVR/SVR ratio after treatment. However, there is limited evidence that PVR/SVR ratio reduced after sildenafil therapy in uncorrected CHD-associated PAH patients. This study aimed to investigate the decreasing of the PVR/SVR ratio after ≥ 1-year oral sildenafil therapy in adult uncorrected CHD-associated PAH. A total of 30 uncorrectable CHD-associated PAH subjects derived from the COHARD-PH registry were included in this study. Right heart catheterization (RHC) was performed during the first visit and further evaluations were conducted after ≥1-year oral sildenafil therapy. The PVR/SVR ratio at the baseline and after the evaluation was collected. The primary outcome of this study was the changes in PVR/SVR ratio from baseline to evaluated RHC. Characteristic analysis of subjects with decreased PVR or PVR/SVR ratio was perforemd as the secondary outcome. The mean PVR and SVR were not different from baseline and evaluated RHC (15.98± 10.67 vs. 18.38±13.93 WU, p=0.206 and 36.65±13.99 vs. 39.34±15.46 WU, p=0.262). There was no significant difference in the baseline PVR/SVR ratio and the evaluated PVR/SVR ratio after ≥1-year sildenafil therapy (0.48 ±0.32 vs. 0.49±0.36; p=0.882). As much as 15 subjects (50%) experienced decreased PVR/SVR ratio. However, there was no significant difference in the characteristics, including age, Eisenmenger syndrome, type of shunts, baseline PVR, PAH-specific treatment, and baseline NT-proBNP level (p>0.05). In conclusion, sildenafil therapy does not change PVR/SVR ratio in adults with uncorrected CHD-associated PAH.

A Case of Successful Cardiopulmonary Resuscitation in a Pregnant Woman with Eisenmengers Syndrome

A Case of Successful Cardiopulmonary Resuscitation in a Pregnant Woman with Eisenmengers Syndrome

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

Heart-Lung Transplantation (HLTX) is required both in primary pulmonary hypertension (PPH) and Eisenmenger syndrome (ES) when there is associated end-stage heart disease. Although PPH is associated with an otherwise structurally normal heart, ES is associated with congenital heart defects, which may increase the complexity of the operation. This study analyzes if the diagnosis (PPH vs. ES) is related to short-term outcomes after HLTX. Patients ≥18 years of age with PPH and ES who underwent HLTX were identified in the United Network for Organ Sharing database from 2005 to 2021. Patients were propensity score matched on heart and lung listing status at the time of transplant. Univariable, multivariable, and Kaplan-Meir survival analyses were performed. The unmatched cohort had 128 PPH and 44 ES patients, and the matched cohort had 44 patients in each group. PPH patients had lower waitlist times and PA pressures but higher FEV1, heart, and lung listing status and ECMO bridge. There were no differences in immediate postoperative outcomes such as dialysis, stroke, and airway dehiscence. PPH patients had a higher treatment rejection in the first year. The 30-day, 1-year, and 3-year survival were better in the PPH group. However, a landmark analysis excluding deaths within 30 days eliminated differences in survival between the groups. Post-transplant dialysis and postoperative mechanical ventilation >5 days were risk factors for 1-year mortality in ES. The short-term outcomes of HLTX are inferior in ES compared to PPH and much of the attrition in ES occurs in the immediate postoperative period.

Pregnancy in Eisenmenger syndrome: a case series from a tertiary care hospital of Northern India.

Despite advances in medical care, we still come across pregnancy in Eisenmenger syndrome. Eisenmenger syndrome represents the severe end of the spectrum for disease in pulmonary artery hypertension associated with CHD. Due to very high maternal and perinatal morbidity and mortality, pregnancy is contraindicated among these women. Current guidelines also recommend that the women who become pregnant should opt for early termination of pregnancy. Here, we present a case series of 11 women of Eisenmenger syndrome and their pregnancy outcome. It was a retrospective analysis of 12 pregnancies among 11 women with Eisenmenger syndrome who were managed in a tertiary care referral centre of Northern India. The mean age of these women was 28 ± 4 years (range 22 to 36 years). Almost 80% of them (9/11) were diagnosed with Eisenmenger syndrome during pregnancy. The commonest cardiac lesion was Ventricular Septal defect (54.5%) followed by Atrial Septal defect (27.3%) and Patent Ductus arteriosus (9.1%). Only three women opted for medical termination of pregnancy, rest eight continued the pregnancy or presented late. Pregnancy complications found include pre-eclampsia (50%), abruption (22%), and fetal growth retardation (62.5%). There were three maternal deaths (mortality rate 27%) in postpartum period. This case series highlights the delay in diagnosis and treatment of CHD despite improvement in medical care. Women with Eisenmenger syndrome require effective contraception, preconceptional counselling, early termination of pregnancy, and multidisciplinary care.

A Rare Case with Dissection of Pulmonary and Aorta in Aortopulmonary Window

Aortopulmonary window is a rare congenital anomaly which is commonly associated with other lesions such as patent ductus arteriosus, interrupted aortic arch, Atrial Septal Defect (ASD), and Ventricular Septal Defect (VSD). Aortic aneurysm and dissection have not been reported as an associated anomaly in AP window. A 44 -year-old male, with inoperable AortoPulmonary Window (AP window) and Eisenmenger syndrome presented to our Emergency Department with back pain and shortness of breath. Transthoracic echocardiography and aortic CT angiography depicted aneurysmal dilatation of ascending aorta with a dissection flap which was extended to main pulmonary artery. Any intervention was very high risk, due to the risk of imminent RV failure. The patient was hemodynamically stable and the symptoms seemed to be chronic, so we decided to manage him medically. AP window is a rare congenital anomaly which is commonly associated with other anomalies. In this report, we represent a rare case of AP window and Eisenmenger syndrome with aortic aneurysm and dissecting flap in ascending aorta and pulmonary artery who was managed medically.

Undiagnosed Case of Ventricular Septal Syndrome with Eisenmenger Syndrome and Epistaxis Presentation

Eisenmenger syndrome is a rare irreversible cardiac condition as a consequence of increased Pulmonary Arterial Hypertension (PAH). This causes a reversal of flow and cyanosis. This pulmonary arterial condition developed because of unrestricted pulmonary flow. It is seen mostly in unrepaired acyanotic congenital cardiac defects with an initial left to right shunt. The common conditions are large Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), Atrioventricular Septal Defect (AVSD), and atrial septal defect. The patient presented with cyanosis and worsening exertional dyspnoea due to the reversal of the shunt. It can be prevented with early diagnosis and by successfully repairing the defect. The mode of diagnosis is a good physical examination and two-dimensional (2D) echocardiography. This case report is about an 11-year-old female patient who presented with a complaint of exertional dyspnoea for five years. She had very low oxygen saturation on room air when presented in the paediatric Outpatient Department (OPD). She went for 2D echocardiography as her physical examination and laboratory investigation were not supporting any diagnosis. Her echocardiography (echo) showed large sub-aortic VSD with Eisenmenger complex and prescribed tablet Sildenafil. On the next day of admission, her dyspnoea improved, and maintained saturation without oxygen of 96-97%. She was successfully discharged and called for regular follow-up.

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P =.0001). Patients achieving 2 or 3 noninva-sive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P =.02; hazard ratio: 4.258, CI: 1.143-15.860, P =.031; hazard ratio: 0.095, CI: 0.013-0.672, P =.018, respectively). Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies.

A rare case of eisenmenger syndrome in a young nigerian male with an undiagnosed atrial septal defect

Eisenmenger syndrome refers to the reversal of an original left-to-right shunt due to long-standing pulmonary hypertension and high pulmonary vascular resistance, in the presence of an intracardiac anatomic defect. Few cases have been reported in Nigeria. The importance of early identification of a cardiac defect cannot be overemphasized, as this leads to avoidance of pulmonary vascular disease (which can impact morbidity and mortality) by repairing the defect. We report a case of an adult male presenting with shortness of breath due to atrial septal defect causing pulmonary arterial hypertension with Eisenmenger physiology, to highlight the occurrence of this rare condition in our locality, to raise awareness for a high index of suspicion.

Anesthesia Management of Sectio Caesarian Patients with Eisenmenger Syndrome and Fetal Distress

Brief overview: 27 year old woman, G2P1A0 gravida 35-36 weeks with congenital heart disease (ventricular septal defect) that progresses to Eisenmenger syndrome, respiratory failure and fetal distress underwent an emergency caesarean section. Management: Anesthesia management was performed under general anesthesia with post-operative care in ICU. Prior to induction, pre-oxygenated with 100% O2 was performed, followed by Rapid Sequence Induction with Ketamine 70 mg, and Rocuronium 50 mg. After the patient had fallen asleep the Sellick maneuver was performed, intubated with ETT no. 6.5. Anesthesia maintenance with Sevoflurane 1 vol%, and 100% oxygen. Fentanyl 50 μg was given after the baby was born. Outcome: In this patient, general anesthetic technique was preferred over regional anesthetic technique for better maternal and fetal outcomes because this patient had already experienced respiratory failure and fetal distress, so a caesarean section was decided as soon as possible. The operation lasted for two hours, with SpO2 during the operation reaching 85% and a live baby boy was born with APGAR scores at the 1st ,3rd and 5th minute 6. Postoperatively, the patient remained intubated and was transferred to the intensive care unit with vital signs blood pressure 122/80 mmHg, pulse 96 beats per minute, SpO2 82-85%.

Characteristics of exercise intolerance in different subgroups of pulmonary arterial hypertension associated with congenital heart disease

BackgroundExercise intolerance is a major manifestation of pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). We aimed to investigate the characteristics of exercise intolerance in different subgroups of PAH-CHD. MethodsWe retrospectively enrolled 171 adult patients with PAH-CHD and 30 age and sex-matched healthy subjects and performed cardiopulmonary exercise testing. Gas exchange parameters, including peak oxygen uptake (peak V̇o2), anaerobic threshold, and the slope of ventilatory equivalent for carbon dioxide (V̇e/V̇co2 slope), were recorded. ResultsThe median age of patients at enrollment was 27.8 years, and 131 (76.6%) were female. Peak V̇o2 was reduced in patients compared to healthy controls (median, 14.8 ml/kg/min versus 26.9 ml/kg/min, p < 0.001). Of all 171 patients, 60 (35.1%) had Eisenmenger syndrome, 35 (20.5%) had PAH associated with systemic-to-pulmonary shunts (PAH-SP), 39 (22.8%) had PAH with small defects (PAH-SD), and 37 (21.6%) had PAH after cardiac defect correction (PAH-CD). Patients with Eisenmenger syndrome had the lowest peak V̇o2 (p = 0.003) and the highest V̇e/V̇co2 slope (p = 0.012), compared with other patients, representing the worst exercise capacity and ventilatory efficiency. Patients with PAH-SP had the best exercise capacity among the four groups, indicated by the highest peak V̇o2 (p = 0.003) compared with other patients. Peak V̇o2 was negatively correlated with pulmonary vascular resistance (r = −0.411, p < 0.001). ConclusionsExercise capacity was severely reduced in patients with PAH-CHD. Among the four subgroups, patients with Eisenmenger syndrome had the worst exercise capacity and ventilatory efficiency.

Pregnancy outcomes of women with Eisenmenger syndrome: A single-center study

BackgroundTo explore the outcomes of mothers with Eisenmenger syndrome (ES) and their offspring. MethodsPregnant women with ES admitted to the Beijing Anzhen Hospital between 2010 and 2019 were retrospectively analyzed and followed up. ResultsForty-two parturient women with ES were recruited, with an average age of 26.7 years (standard deviation [SD], ±4.0 years). The average gestational age was 33.7 weeks (SD, ±2.5 weeks). The average percutaneous oxygen saturation was 84.1 (±9.2), and 40 (95.2%) had caesarean delivery. The average pulmonary artery systolic pressure was 107.5 mmHg (SD, ±20.3 mmHg). Twelve (28.6%) women experienced pulmonary hypertensive crisis; 11 (26.2%) of these women died. Regarding the offspring, the average fetal weight was 1778.1 g (SD, ±555.3 g), six (14.3%) died, and congenital heart disease was diagnosed in three (7.1%). There were significant differences in age, gestational age, percutaneous oxygen saturation, Apgar score, and heart failure between the maternal death and non-death groups (P < 0.05). Death was mainly related to pulmonary hypertensive crisis and heart failure. ConclusionsWe recommend pregnancy termination if ES occurs during early pregnancy; however, patients should be informed of the risks if it occurs during late pregnancy. Multidisciplinary cooperation should be strengthened to improve the prognosis of the mothers and their offspring.

Partial anomalous pulmonary venous return in adults: Insight into pulmonary hypertension

BackgroundPartial anomalous pulmonary venous return (PAPVR) is a rare condition that may lead to pulmonary arterial hypertension (PAH). We sought to determine the prevalence of PAPVR, the follow-up rate of incidentally discovered PAPVR, the repercussions of volume and pressure overload on the right ventricle (RV), and the prevalence and predictors of PAH. Methods and resultsFifty PAPVR patients aged ≥18 years were included. All underwent transthoracic echocardiography and multislice imaging. PAPVR prevalence was 0.2% (23/13,606 chest computed tomography reports). Of the 26 incidentally diagnosed patients, 14 (54%) did not have a cardiac follow-up. Seven (14%) patients had PAH, of whom one third (28%) had Eisenmenger syndrome. The left-to-right shunt ratio (Qp/Qs) was the only predictor of PAH. PAPVR-induced volume overload led to increased right chamber volumes (RV basal diameter 45±8 mm, RV mid-diameter 38±9 mm, RV/left ventricle ratio 1.2 ± 0.2, indexed RV end-diastolic area 14±4 cm2/m2, indexed RV end-systolic area 8.6 ± 3.2 cm2/m2) and increased RV functional parameters (tricuspid annular plane systolic excursion 27±5 mm, fractional area change [FAC] 42 ± 10%, global longitudinal strain [GLS] −22 ± 5). In contrast, PAH-induced pressure overload had no impact on right heart volumes, but on RV functional parameters, which were decreased compared to non-PAH patients (FAC 35 ± 13% vs. 43 ± 10%, p = 0.049; GLS -18 ± 5 vs. −23 ± 4%, p = 0.01). ConclusionsPAPVR prevalence was 0.2%. When incidentally diagnosed, its management was often neglected despite potentially serious consequences (14% PAH). Only the Qp/Qs ratio was predictive of PAH. PAPVR-induced volume overload was shown to increase right heart volumes, while PAH-induced pressure overload caused alterations of RV functional parameters.

Survival of patients with congenital ventricular septal defect.

The long-term survival of patients with isolated congenital ventricular septal defect (VSD) is not well described. The aim of this study was to describe the survival of a national cohort of patients with VSD compared with the general population. Using Danish nationwide medical registries, all patients diagnosed with congenital VSD (n = 9,136) in the period 1977-2018 were included. Patients with chromosomal abnormalities and concomitant congenital cardiac malformations other than atrial septal defect were excluded. Each patient was matched by birthyear and sex with ten controls from the general Danish population. Kaplan-Meier survival function and Cox proportional hazard regression were used to compute survival and mortality risk. Median follow-up was 22 years (interquartile range: 11-37). VSD patients displayed lower survival (P<0.001) yielding a hazard ratio (HR) for mortality of 2.7 [95% confidence interval (CI): 2.4-3.0] compared with matched controls. The adjusted HR for mortality among patients with unrepaired VSD was 2.7 (95% CI: 2.4-3.0) and 2.8 (95% CI: 2.1-3.7) for patients with surgically closed VSD. Stratified by era of VSD diagnosis, the HR for mortality was 3.2 (95% CI: 2.8-3.7) for unrepaired patients diagnosed before 1990 and 2.4 (95% CI: 2.0-2.7) for patients diagnosed later. Cardiac-related death was the commonest cause of death among unrepaired (30%) and surgically closed (65%) patients. Patients with VSD had lower survival compared with the general population. The HR for mortality was increased over 2.5-fold in patients with unrepaired defect (Eisenmenger syndrome excluded) and over 1.5-fold in patients with surgically closed defect (excluding surgical mortality).