-
JACC. Case Reports Jun 2022A patient diagnosed with expanded Goldenhar complex with oculoauriculovertebral spectrum complicated with complex pulmonary and congenital heart disease, underwent...
A patient diagnosed with expanded Goldenhar complex with oculoauriculovertebral spectrum complicated with complex pulmonary and congenital heart disease, underwent successful heart-lung transplantation 21 years ago, with excellent functional outcome and good quality of life. Heart-lung transplantation can be an option of care for patients with expanded Goldenhar complex. ().
PubMed: 35734534
DOI: 10.1016/j.jaccas.2022.05.001 -
Medicina (Kaunas, Lithuania) Mar 2022: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal...
Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension-A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals.
: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients' well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. : The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. : Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. : On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes.
Topics: Abortion, Spontaneous; Adult; Cesarean Section; Delivery of Health Care; Eisenmenger Complex; Familial Primary Pulmonary Hypertension; Female; Fetus; Heart Defects, Congenital; Humans; Infant, Newborn; Pregnancy; Pregnant Women; Pulmonary Arterial Hypertension; Retrospective Studies
PubMed: 35454315
DOI: 10.3390/medicina58040476 -
BMC Cardiovascular Disorders Mar 2022Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes...
BACKGROUND
Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH.
CASE PRESENTATION
A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis.
CONCLUSIONS
Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.
Topics: Adult; Angina Pectoris; Coronary Angiography; Eisenmenger Complex; Humans; Hypertension, Pulmonary; Male; Pulmonary Artery; Quality of Life; Syndrome
PubMed: 35247981
DOI: 10.1186/s12872-022-02524-w -
Heart (British Cardiac Society) Jan 2020The optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific... (Comparative Study)
Comparative Study
OBJECTIVE
The optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific morbidity after heart-lung transplantation (HLTx) or lung transplantation (LTx) in a cohort of Nordic patients with ES to aid decision-making for scheduling transplantation.
METHODS
We performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012.
RESULTS
Among 714 patients with ES in the Nordic region, 63 (9%) underwent transplantation. The median age at transplantation was 31.9 (IQR 21.1-42.3) years. Within 30 days after transplantation, seven patients (11%) died. The median survival was 12.0 (95% CI 7.6 to 16.4) years and the overall 1-year, 5-year, 10-year and 15-year survival rates were 84.1%, 69.7%, 55.8% and 40.6%, respectively. For patients alive 1 year post-transplantation, the median conditional survival was 14.8 years (95% CI 8.0 to 21.8), with 5-year, 10-year and 15-year survival rates of 83.3%, 67.2% and 50.0%, respectively. There was no difference in median survival after HLTx (n=57) and LTx (n=6) (14.9 vs 10.6 years, p=0.718). Median cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis/kidney transplantation-free survival rates were 11.2 (95% CI 7.8 to 14.6), 6.9 (95% CI 2.6 to 11.1) and 11.2 (95% CI 8.8 to 13.7) years, respectively. The leading causes of death after the perioperative period were infection (36.7%), bronchiolitis obliterans syndrome (23.3%) and heart failure (13.3%).
CONCLUSIONS
This study shows that satisfactory post-transplantation survival, comparable with contemporary HTx and LTx data, without severe comorbidities such as cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis, is achievable in patients with ES, with a conditional survival of nearly 15 years.
Topics: Adolescent; Adult; Child; Clinical Decision-Making; Decision Support Techniques; Eisenmenger Complex; Female; Heart-Lung Transplantation; Humans; Lung Transplantation; Male; Patient Selection; Postoperative Complications; Predictive Value of Tests; Retrospective Studies; Risk Assessment; Risk Factors; Scandinavian and Nordic Countries; Time Factors; Time-to-Treatment; Treatment Outcome; Young Adult
PubMed: 31434713
DOI: 10.1136/heartjnl-2019-315345 -
Hellenic Journal of Cardiology : HJC =... 2023Data regarding the prognosis of Eisenmenger syndrome (ES) and effect of targeted drugs are limited. This study aimed to analyze the prognosis and impact of targeted drug...
BACKGROUND
Data regarding the prognosis of Eisenmenger syndrome (ES) and effect of targeted drugs are limited. This study aimed to analyze the prognosis and impact of targeted drug therapy on the survival rate of patients with ES in the Chinese population.
METHODS
The data of patients with ES referred to our hospital between January 2010 and December 2020 were retrospectively analyzed. Data included baseline demographics, echocardiographic parameters, and clinical diagnoses. All patients were followed up via telephone interviews in February 2022. The primary endpoint was mortality.
RESULTS
Overall, 1,021 patients with ES were included. The 1-, 3-, 5-, 7-, 10-, and 12-year survival rates were 91.6%, 84.2%, 80.7%, 73.8%, 71.4%, and 69.9%, respectively. Patients with atrial septal defects had the best prognosis than those with ventricular septal defects, patent ductus arteriosus, and complex congenital heart disease (CHD) (P < 0.0001). Patients who visited between 2016 and 2020 received increased targeted drug therapy and had a better prognosis than those who visited between 2010 and 2015 (all P < 0.05). Cox regression analysis revealed age, pulmonary arterial systolic pressure, post-tricuspid shunt CHD, targeted drugs, and year of the first hospital visit to be predictors of death (P < 0.05).
CONCLUSIONS
Survival rates associated with an increased use of combined targeted drugs significantly improved in patients with ES. However, numerous factors that predict increased mortality remain to be elucidated.
Topics: Humans; Eisenmenger Complex; Retrospective Studies; Prognosis; Heart Septal Defects, Ventricular; Heart Septal Defects, Atrial
PubMed: 36924996
DOI: 10.1016/j.hjc.2023.03.004 -
Transplant International : Official... Jul 2019Transplant type for end-stage pulmonary vascular disease remains debatable. We compared recipient outcome after heart-lung (HLT) versus double-lung (DLT)... (Comparative Study)
Comparative Study
Transplant type for end-stage pulmonary vascular disease remains debatable. We compared recipient outcome after heart-lung (HLT) versus double-lung (DLT) transplantation. Single-center analysis (38 HLT-30 DLT; 1991-2014) for different causes of precapillary pulmonary hypertension (PH): idiopathic (22); heritable (two); drug-induced (nine); hepato-portal (one); connective tissue disease (four); congenital heart disease (CHD) (24); chronic thromboembolic PH (six). HLT decreased from 91.7% [1991-1995] to 21.4% [2010-2014]. Re-intervention for bleeding was higher after HLT; (P = 0.06) while primary graft dysfunction grades 2 and 3 occurred more after DLT; (P < 0.0001). Graft survival at 90 days, 1, 5, 10, and 15 years was 93%, 83%, 70%, 47%, and 35% for DLT vs. 82%, 74%, 61%, 48%, and 30% for HLT, respectively (log-rank P = 0.89). Graft survival improved over time: 100%, 93%, 87%, 72%, and 72% in [2010-2014] vs. 75%, 58%, 42%, 33%, and 33% in [1991-1995], respectively; P = 0.03. No difference in chronic lung allograft dysfunction (CLAD)-free survival was observed: 80% & 28% for DLT vs. 75% & 28% for HLT after 5 and 10 years, respectively; P = 0.49. Primary graft dysfunction in PH patients was lower after HLT compared to DLT. Nonetheless, overall graft and CLAD-free survival were comparable and improved over time with growing experience. DLT remains our preferred procedure for all forms of precapillary PH, except in patients with complex CHD.
Topics: Adolescent; Adult; Child; Child, Preschool; Connective Tissue Diseases; Disease-Free Survival; Female; Graft Survival; Heart Defects, Congenital; Heart-Lung Transplantation; Hospital Mortality; Humans; Lung Transplantation; Male; Middle Aged; Postoperative Complications; Preoperative Period; Primary Graft Dysfunction; Pulmonary Arterial Hypertension; Retrospective Studies; Thromboembolism; Young Adult
PubMed: 30735591
DOI: 10.1111/tri.13409 -
Journal of the American Heart... May 2024Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However,...
BACKGROUND
Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However, the risk stratification for persistent pulmonary hypertension (PH) in this population remains uncertain.
METHODS AND RESULTS
We retrospectively enrolled 103 patients with pretricuspid shunts with high total pulmonary resistance >4.5 Wood units (estimated pulmonary vascular resistance ≥3 Wood units). During a mean±SD follow-up of 20.95±24.84 months, 32 patients developed postoperative persistent PH after shunt correction. We identified 3 significant predictors of postoperative persistent PH, including mean pulmonary artery pressure after inhaled oxygen ≥40.5 mm Hg (odds ratio [OR], 7.78 [95% CI, 2.02-30.03]; <0.01), total pulmonary resistance after inhaled oxygen ≥6.5 Wood units (estimated pulmonary vascular resistance ≥5 Wood units; OR, 12.23 [95% CI, 2.12-70.46]; <0.01), and artery oxygen saturation at rest <95% (OR, 3.34 [95% CI, 1.07-10.44]; =0.04). We established the prediction model with the C-statistics of 0.85 (95% CI, 0.77-0.93; <0.01), and the C-statistic was 0.83 (95% CI, 0.80-0.86) after bootstrapping 10 000 times with a good performance of the nomogram calibration curve for predicting persistent PH.
CONCLUSIONS
Our study presents a multivariable risk stratification model for persistent PH after shunt correction in adults with pretricuspid shunts. This model, based on 3 hemodynamic predictors after inhaled oxygen, may assist in identifying individuals at higher risk of persistent PH after shunt correction.
Topics: Humans; Female; Male; Hypertension, Pulmonary; Retrospective Studies; Nomograms; Adult; Vascular Resistance; Risk Assessment; Pulmonary Artery; Middle Aged; Risk Factors; Predictive Value of Tests; Treatment Outcome; Cardiac Surgical Procedures; Heart Defects, Congenital; Arterial Pressure
PubMed: 38639332
DOI: 10.1161/JAHA.123.032412 -
Medicina (Kaunas, Lithuania) Sep 2021Mirror syndrome (MS) or Ballantyne's syndrome is a rare maternal condition that can be life-threatening for both mother and fetus. The condition is characterized by...
Mirror syndrome (MS) or Ballantyne's syndrome is a rare maternal condition that can be life-threatening for both mother and fetus. The condition is characterized by maternal signs and symptoms similar to those seen in preeclampsia in the setting of fetal hydrops. Despite recent advances in the field of maternal-fetal medicine, the etiopathogenesis of MS remains elusive. For patients and doctors, the COVID-19 pandemic has become an extra hurdle to overcome. The following case illustrates how patients' non-compliance associated with mirror syndrome and SARS-CoV-2 infection led to the tragic end of a 19-year-old patient. Therefore, knowledge of the signs and symptoms of mirror syndrome should always be part of the armamentarium of every obstetrician.
Topics: Adult; COVID-19; Eisenmenger Complex; Female; Humans; Hydrops Fetalis; Pandemics; Pregnancy; SARS-CoV-2; Young Adult
PubMed: 34684068
DOI: 10.3390/medicina57101031 -
Indian Pacing and Electrophysiology... 202231 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome,...
31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Transcatheter leadless pacemaker was deployed in her RV septum despite some unforeseen technical problems. This patient had intrahepatic interruption of IVC with Azygous continuation draining into SVC but this altered venovascular course was detected only fluoroscopically midway during the pacemaker implantation procedure and this was not detected in the preprocedural transthoracic echocardiography. This abnormal venous course was clearly demonstrated in the cardiac CT which was performed only after completion of the pacemaker implantation procedure in this patient. The technical challenges encountered mainly were mostly during the manipulation of the 27F delivery catheter of Micra through this altered cardiovascular anatomy via transfemoral approach and also due to the presence of septal defects. Thus, transcatheter leadless permanent pacemaker was implanted successfully through transfemoral access in this complex congenital heart disease with interrupted IVC and azygous continuation. Besides transthoracic echocardiography, it may be better to perform transesophageal echocardiography or even preferably radiological imaging like cardiac CT or MRI prior to transcatheter leadless pacemaker implantation in patients with complex congenital heart disease to understand the cardiovascular anatomy and plan the procedure.
PubMed: 35364233
DOI: 10.1016/j.ipej.2022.03.003 -
Frontiers in Cardiovascular Medicine 2024A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to...
Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.
AIMS
A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA).
METHODS AND RESULTS
Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed. Patients were classified according to predefined ABCDE categories (A: Eisenmenger syndrome, B: left-to-right shunt, C: coincidental defects, including all ASDs, D: corrected CHD, E: TGA), or as complex CHD (group 5), by 2 independent investigators. In case of disagreement, a third reviewer could either settle a final decision, or the patient was deemed not classifiable. Survival curves were calculated for each group and compared to idiopathic PAH patients of the registry. A total of 223 out of 531 patients in the registry had PAH-CHD, and 193 were categorized to the following groups: A 39(20%), B 27(14%), C 62(32%) including 43 ASDs, D 58(30%), E 7(4%), whereas 6 patients were categorized as group 5, and 10 patients were unable to be classified. No survival difference could be demonstrated between the groups.
CONCLUSIONS
This modified classification seems to be more applicable to pediatric PAH-CHD patients than the previous classification, but some patients with PAH-CHD who never had a shunt remain unclassifiable. The role of ASD in pediatric PH should be reconsidered.
PubMed: 38370158
DOI: 10.3389/fcvm.2024.1344014