-
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 -
Congenital Heart Disease 2015Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not...
BACKGROUND
Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease.
METHODS
Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029.
RESULTS
Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot.
CONCLUSIONS
Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.
Topics: Adolescent; Adult; Female; Forecasting; Heart Defects, Congenital; Humans; Male; Middle Aged; Survival Rate; Time Factors; Young Adult
PubMed: 25043406
DOI: 10.1111/chd.12201 -
Multimedia Manual of Cardiothoracic... 2014It is axiomatic that those performing surgery on the congenitally malformed heart require a thorough knowledge of the lesions they will be called upon to correct. The...
It is axiomatic that those performing surgery on the congenitally malformed heart require a thorough knowledge of the lesions they will be called upon to correct. The necessary anatomical knowledge is becoming increasingly difficult to obtain at first hand, since relatively few centres now hold archives of specimens obtained in an appropriately legal fashion from the patients unfortunately dying in previous years. One centre with such an archive is Ann and Robert H. Lurie Children's Hospital in Chicago, known previously as Chicago Memorial Children's Hospital. The archive was established by Farouk S. Idriss, and was subsequently enhanced and consolidated by his son, Rachid. It is now under the care of Carl L. Backer, the current chief of paediatric cardiothoracic surgery at Lurie Children's. With the support of Carl, the archive has been triaged and catalogued by Diane E. Spicer and Robert H. Anderson. It has now been used to create a series of video presentations, illustrating the salient features of surgical anatomy of selected entities, with the videoclips being edited and prepared for publication by Anne Sarwark. This video contains the fruits of the first of these exercises in anatomy, and is devoted to tetralogy of Fallot.We begin the exercise by making comparisons between the normal heart and the arrangement seen in typical tetralogy. We emphasize the need to recognize the 'building blocks' of the normal outflow tracts, and show how they come apart in tetralogy. We then show the variations to be found in the specific morphology of the borders of the hole between the ventricles, with the crest of the apical ventricular septum being overridden by the orifice of the aortic valve such that the latter structure has a biventricular connection. We emphasize that it is the squeeze between the deviated muscular outlet septum and septoparietal trabeculations that is the essential phenotypic feature of the lesion. We then proceed to demonstrate the further variation to be found in the length of the outlet septum, which in extreme cases can be fibrous and hypoplastic rather than muscular. We also show how the ventriculo-arterial connection can vary from being concordant to becoming double outlet from the right ventricle. We conclude by emphasizing that the anatomy of tetralogy can also be recognized when the subpulmonary outflow tract is atretic rather than stenotic.
Topics: Audiovisual Aids; Cardiology; Child; Databases as Topic; Eisenmenger Complex; Heart Ventricles; Humans; Imaging, Three-Dimensional; Tetralogy of Fallot
PubMed: 25500768
DOI: 10.1093/mmcts/mmu024 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2018In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and...
BACKGROUND
In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and long-term outcomes.
METHODS
We retrospectively reviewed the medical records of a total of 30 patients who were followed with the diagnosis of aortopulmonary window in our hospital between May 1998 and June 2016. The clinical characteristics of the patients, echocardiographic and angiographic findings, surgical treatment outcomes, and medical problems during follow-up were reviewed.
RESULTS
The most common signs and symptoms were murmur, dyspnea, tachypnea, growth retardation, and signs of congestive heart failure. The mean age at the time of surgery was 8.2±14.4 months (7 days to 60 months). Eighteen patients (60%) had additional congenital cardiac anomalies. Eleven patients had simple congenital heart diseases, and seven patients had complex congenital heart diseases. Four patients were unable to be operated due to Eisenmenger syndrome (n=3) and complex congenital heart disease (n=1). No early or late postoperative death was observed. The mean follow-up was 6.4±4.8 years (range, 5 months to 16 years). In addition to aortopulmonary window repair, an additional cardiac anomaly modifying surgical intervention was corrected in nine patients (34.6%). One patient was reoperated for residual aortopulmonary window and another patient for pulmonary stenosis (valvular, supravalvar) after three years. One of these patients underwent pulmonary balloon valvuloplasty after two years. The reoperation rate was 7.7% (n=2) during follow-up.
CONCLUSION
Aortopulmonary window is a rare cardiac anomaly which may be overlooked by echocardiographic study, and which is amenable for repair with low-surgical risk. It is, therefore, imperative to diagnose and treat this condition, before pulmonary vascular disease develops.
PubMed: 32082708
DOI: 10.5606/tgkdc.dergisi.2018.14772 -
Acta Cardiologica Sinica Nov 2015Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH... (Review)
Review
Insight into Pulmonary Arterial Hypertension Associated with Congenital Heart Disease (PAH-CHD): Classification and Pharmacological Management from a Pediatric Cardiological Point of View.
UNLABELLED
Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH classification does not completely characterize or individualize any subgroup of pediatric PH. This is in contrast to the Panama classification, in which prenatal and fetal origins of many pulmonary vascular diseases in neonates and children, perinatal pulmonary vascular maladaptation, prenatal and postnatal pulmonary vascular mal-development, and pulmonary vascular hypoplasia are included. Currently, the updated treatment algorithm for adults with pulmonary arterial hypertension (PAH), including PAH associated with congenital heart disease (PAH-CHD) and idiopathic PAH, etc. has been reported. It has been suggested to treat FC III patients with Eisenmenger syndrome (ES) with bosentan. However, there is no evidence-based treatment algorithm for children with PAH-CHD. Moreover, it is necessary to develop a more comprehensive algorithm in which multiple specific pediatric risk factors are determined, and the critical goal of treatment should be to permit normal activities without the need to self-limit in children with PAH-CHD. Together, the beneficial data on specific-target pharmacologic interventions are still quite preliminary, and large trials are warranted. Specifically, the extrapolation of the other forms of the disease, such as ES, should be undertaken carefully.
KEY WORDS
Congenital heart disease; Eisenmenger syndrome; Pulmonary arterial hypertension; Target therapy.
PubMed: 27122915
DOI: 10.6515/acs20150424b -
Brain Pathology (Zurich, Switzerland) Nov 2015Huntington's disease (HD), an autosomal dominantly inherited polyglutamine or CAG repeat disease along with somatomotor, oculomotor, psychiatric and cognitive symptoms,...
Huntington's disease (HD), an autosomal dominantly inherited polyglutamine or CAG repeat disease along with somatomotor, oculomotor, psychiatric and cognitive symptoms, presents clinically with impairments of elementary and complex visual functions as well as altered visual-evoked potentials (VEPs). Previous volumetric and pathoanatomical post-mortem investigations pointed to an involvement of Brodmann's primary visual area 17 (BA17) in HD. Because the involvement of BA17 could be interpreted as an early onset brain neurodegeneration, we further characterized this potential primary cortical site of HD-related neurodegeneration neuropathologically and performed an unbiased estimation of the absolute nerve cell number in thick gallocyanin-stained frontoparallel tissue sections through the striate area of seven control individuals and seven HD patients using Cavalieri's principle for volume and the optical disector for nerve and glial cell density estimations. This investigation showed a reduction of the estimated absolute nerve cell number of BA17 in the HD patients (71,044,037 ± 12,740,515 nerve cells) of 32% in comparison with the control individuals (104,075,067 ± 9,424,491 nerve cells) (Mann-Whitney U-test; P < 0.001). Additional pathoanatomical studies showed that nerve cell loss was most prominent in the outer pyramidal layer III, the inner granular layers IVa and IVc as well as in the multiform layer VI of BA17 of the HD patients. Our neuropathological results in BA17 confirm and extend previous post-mortem, biochemical and in vivo neuroradiological HD findings and offer suitable explanations for the elementary and complex visual dysfunctions, as well as for the altered VEP observed in HD patients.
Topics: Adult; Aged; Cell Count; Female; Humans; Huntington Disease; Male; Middle Aged; Nerve Degeneration; Neuroglia; Neurons; Visual Cortex
PubMed: 25495445
DOI: 10.1111/bpa.12237 -
Heart (British Cardiac Society) Jan 2018Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with...
OBJECTIVES
Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome.
METHODS
Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years.
RESULTS
Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×10/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×10/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively).
CONCLUSIONS
Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.
Topics: Adult; Blood Platelets; Eisenmenger Complex; Female; Follow-Up Studies; Forecasting; Humans; Male; Mean Platelet Volume; Platelet Count; Retrospective Studies; Risk Factors; Survival Rate; Thrombocytopenia; United Kingdom
PubMed: 28663364
DOI: 10.1136/heartjnl-2016-311144 -
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