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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 -
Journal of the American Heart... Mar 2020Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We...
Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We sought to define the predictors of SCD in this population. Methods and Results A retrospective analysis of all patients with Eisenmenger syndrome from 2 large tertiary referral centers was performed. ECGs, prolonged ambulatory recordings, echocardiograms, and clinical histories were reviewed; and the cause of death was identified. A total of 246 patients (85 [34.6%] men) with a mean age of 37.3 (±14.2) years were followed up for a median of 7 years. Over the study period, 136 patients died, with 40 experiencing SCD and 74 experiencing cardiac death (sudden and nonsudden). Age, atrial fibrillation, prolonged QRS duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased risk of SCD, whereas advanced pulmonary hypertension therapies were protective. Atrial fibrillation (11.45-fold increased risk; <0.001) and QRS duration ≥120 ms (2.06-fold increased risk; =0.034) remained significant predictors of SCD in the multivariate analysis, whereas advanced pulmonary hypertension therapies were strongly protective against SCD (<0.001). Conclusions Atrial arrhythmias, impaired ventricular function, and conduction system disease were associated with increased risk of SCD in this cohort of patients with Eisenmenger syndrome, providing an opportunity for early risk stratification and potential intervention. Clinical heart failure symptoms (New York Heart Association class ≥II) were predictive of increased mortality but not of SCD, suggesting a potential arrhythmic cause behind SCD.
Topics: Adult; Arrhythmias, Cardiac; Cause of Death; Death, Sudden, Cardiac; Eisenmenger Complex; Female; Florida; Humans; Los Angeles; Male; Middle Aged; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Young Adult
PubMed: 32174228
DOI: 10.1161/JAHA.119.014554 -
Cardiovascular Pathology : the Official... 1996Reports on heart-lung transplantation emphasize the pathology of the transplanted lungs. This study is a clinicopathological assessment of cardiac pathology in the...
Reports on heart-lung transplantation emphasize the pathology of the transplanted lungs. This study is a clinicopathological assessment of cardiac pathology in the hearts transplanted as part of the combined heart-lung block. Seventy-five consecutive heart-lung transplants (H-LTx) performed between 1981 and 1989 were studied. Endomyocardial biopsy, autopsy and clinical data were analyzed for information on cardiac rejection, graft coronary disease, transplant survival and the presence of obliterative bronchiolitis and compared with controls. The controls consisted of 391 heart transplants (HTx) performed in 361 recipients over the same time period. Sixty-three adults and nine children received H-LTx (48.6% male; 51.4% female). In this study, H-LTx were performed primarily for Eisenmenger's complex (33 72 ) and primary pulmonary hypertension (28 72 ). At 1 year H-LTx survival was 63.88%, versus 81.54% in HTx alone and 63.63% in lung transplant recipients without heart grafts. The results showed that H-LTx patients have less cardiac rejection compared to patients who undergo HTx alone (p < .005). Only 40% of H-LTx recipients developed acute cardiac rejection in the initial 3 months posttransplantation (post-Tx), compared with 80% of HTx controls. Only 4% of H-LTx recipients developed cardiac rejection after the initial 6 months. No cardiac rejection was observed in the initial 5 years post-Tx in 49.9% of H-LTx. Graft coronary disease was seen in 7.73% of H-LTx within the first 5 years post-Tx compared with 25.87% in HTx recipients alone (p < .005). Obliterative bronchiolitis (OB) was present in 71.43% of H-LTx with graft coronary disease, compared to OB in only 41.38% of H-LTx without graft coronary disease (p < .05). In conclusion, H-LTx recipients have less acute cardiac rejection episodes than HTx recipients alone (p < .005). Most cardiac rejection in H-LTx occurs within the initial 6 months. In addition, H-LTx recipients develop less graft coronary disease than HTx recipients (p < .005). Obliterative bronchiolitis and graft coronary disease may be the result of the same immunological process, as 71.43% of H-LTx with graft coronary disease also had OB. Survival for H-LTx patients is more similar to that of lung transplant (LTx) patients alone than that of heart transplants alone, suggesting that it is the pulmonary pathology portion, rather than the cardiac pathology portion, in combined H-LTx transplants that contributes more to H-LTx survival.
PubMed: 25851477
DOI: 10.1016/1054-8807(95)00114-x -
The Canadian Journal of Cardiology Nov 2006It has long been debated whether patients with atrial septal defect (ASD) Eisenmenger syndrome have idiopathic pulmonary arterial hypertension with an incidental ASD or...
BACKGROUND
It has long been debated whether patients with atrial septal defect (ASD) Eisenmenger syndrome have idiopathic pulmonary arterial hypertension with an incidental ASD or severe pulmonary hypertension on the basis of their ASD shunt magnitude alone.
HYPOTHESIS
It was hypothesized that if ASD Eisenmenger patients had idiopathic pulmonary arterial hypertension with an incidental ASD, a mutation in the bone morphogenetic protein receptor-2 (BMPR2) would be found in some of these patients.
PATIENTS AND METHODS
All adult patients with ASD Eisenmenger syndrome were identified from the databases of two adult congenital cardiac units, and were matched to a control group with similar types of ASDs and no pulmonary hypertension. Gene coding for BMPR2 was examined for mutation using denaturing high-performance liquid chromatography of the entire coding sequence.
RESULTS
Eighteen adult patients with ASD Eisenmenger syndrome and 18 control patients were identified. ASD Eisenmenger patients had significantly larger ASDs than the control patients (3.7+/-1.2 cm versus 1.9+/-0.7 cm, P<0.01). A mutation in BMPR2 was not detected in either group.
CONCLUSION
ASD Eisenmenger syndrome may occur without BMPR2 mutation. Whether shunt magnitude alone or in combination with yet another genetic mutation is responsible for the development of pulmonary hypertension in these patients remains to be determined.
Topics: Adult; Bone Morphogenetic Protein Receptors, Type II; Case-Control Studies; DNA Primers; Eisenmenger Complex; Exons; Female; Genetic Predisposition to Disease; Heart Septal Defects, Atrial; Humans; Hypertension, Pulmonary; Introns; Male; Middle Aged; Mutation; Ontario; Pulmonary Wedge Pressure; Quebec; Sequence Analysis, DNA; Transcription, Genetic
PubMed: 17102831
DOI: 10.1016/s0828-282x(06)70950-3 -
Revista Portuguesa de Cardiologia May 2018The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET).
AIM
The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET).
METHODS
A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction.
RESULTS
We analyzed 154 CPET cases. There were significant differences between groups, with the lowest peak oxygen consumption (VO) values seen in patients with cardiac shunts (39% with Eisenmenger physiology) (17.2±7.1ml/kg/min, compared to 26.2±7.0ml/kg/min in tetralogy of Fallot patients; p<0.001), the lowest percentage of predicted peak VO in complex heart defects (50.1±13.0%) and the highest minute ventilation/carbon dioxide production slope in cardiac shunts (38.4±13.4). Chronotropism was impaired in patients with complex defects. Eisenmenger syndrome (n=17) was associated with the lowest peak VO (16.9±4.8 vs. 23.6±7.8ml/kg/min; p=0.001) and the highest minute ventilation/carbon dioxide production slope (44.8±14.7 vs. 31.0± 8.5; p=0.002). Age, cyanosis, CPET duration, peak systolic blood pressure, time to anaerobic threshold and heart rate at anaerobic threshold were predictors of the combined outcome of all-cause mortality and hospitalization for cardiac cause.
CONCLUSION
Across the spectrum of CHD, cardiac shunts (particularly in those with Eisenmenger syndrome) and complex defects were associated with lower functional capacity and attenuated chronotropic response to exercise.
Topics: Adult; Exercise Test; Female; Heart Defects, Congenital; Heart Diseases; Humans; Male; Retrospective Studies
PubMed: 29776810
DOI: 10.1016/j.repc.2017.09.020 -
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 -
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 -
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