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Homoarginine-A prognostic indicator in adolescents and adults with complex congenital heart disease?PloS One 2017Homoarginine (hArg) has been shown to be of prognostic value in patients with chronic left heart failure. The present study aims to assess the clinical utility and...
BACKGROUND
Homoarginine (hArg) has been shown to be of prognostic value in patients with chronic left heart failure. The present study aims to assess the clinical utility and prognostic value of hArg levels in patients with complex congenital heart disease (CHD).
METHODS
Plasma hArg levels were measured in 143 patients with complex CHD and compared to clinical status, echocardiographic and laboratory parameters as well as the occurrence of adverse cardiac events.
RESULTS
Median hArg levels were 1.5 μmol/l in CHD patients as compared to 1.70 μmol/l in healthy controls (p = 0.051). Median hArg levels were lowest in patients with Fontan palliation (1.27 μmol/l) and Eisenmenger physiology (0.99 μmol/l) and decreased with the severity of adverse cardiac events with lowest values found in patients prior to death or overt heart failure (0.89 μmol/l). According to ROC analysis, the most important predictors of adverse cardiac events were hArg levels (AUC 0.837, p<0.001, CI 0.726-0.947), NYHA class (AUC 0.800, p<0.001, CI 0.672-0.928) and NT-proBNP levels (AUC 0.780, p<0.001, CI 0.669-0.891). The occurrence of overt heart failure or death due to progressive heart failure were best predicted by NYHA class (AUC 0.945, p<0.001, CI 0.898-0.992), hArg levels (AUC 0.911, p<0.001, CI 0.850-0.971) and NT-proBNP levels (AUC 0.877, p<0.001, CI 0.791-0.962), respectively.
CONCLUSION
In patients with complex CHD, hArg levels can predict adverse cardiac events as reliably as or even better than NT-proBNP levels and thus might be of prognostic value in this subset of patients.
Topics: Adolescent; Adult; Biomarkers; Echocardiography; Female; Heart Defects, Congenital; Homoarginine; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Prognosis; ROC Curve
PubMed: 28886170
DOI: 10.1371/journal.pone.0184333 -
Beijing Da Xue Xue Bao. Yi Xue Ban =... Dec 2014To investigate the clinical presentation, diagnosis and treatment of the Eisenmenger's syndrome in pregnancy. (Review)
Review
OBJECTIVE
To investigate the clinical presentation, diagnosis and treatment of the Eisenmenger's syndrome in pregnancy.
METHODS
One case of Eisenmenger's syndrome in pregnancy in our hospital was retrospectively analyzed and the literature reviewed.
RESULTS
This patient who received antenatal care irregularly in other hospital was diagnosed with Eisenmenger's syndrome with the symptoms of chest distress. The other hospital suggested her to terminate pregnancy, but the patient refused. She was admitted to our hospital in her late pregnancy, received the treatment of rest, oxygen, blood gas and pulmonary artery pressure monitoring. Considering perioperative pulmonary hypertension crisis likely to occur, pulmonary artery catheter was placed preoperatively. In general anesthesia, the cesarean was performed. After operation, the patient was transferred to the intensive care unit, with the treatment of expanding blood vessels, reducing pulmonary artery pressure, administering anticoagulation and preventing infection. One week later, the patient was discharged from hospital with smooth condition.
CONCLUSION
Eisenmenger's syndrome in pregnancy is associated with extremely poor maternal and fetal outcome. Termination of pregnancy should be offered to such patients. When interruption of pregnancy is refused, the early hospital admission is needed. The patients should be managed in a high-risk pregnancy unit by a multidisciplinary team from obstetrics, cardiology, anesthesia and pediatrics for improving pregnancy outcomes.
Topics: Anesthesia, General; Eisenmenger Complex; Female; Humans; Hypertension, Pulmonary; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome
PubMed: 25512298
DOI: No ID Found -
Revista Portuguesa de Cardiologia :... Feb 2013Bosentan is recommended for symptomatic patients with Eisenmenger syndrome due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However,...
BACKGROUND
Bosentan is recommended for symptomatic patients with Eisenmenger syndrome due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However, its long-term efficacy and safety in patients with pulmonary arterial hypertension (PAH) associated with complex congenital heart disease (CHD) is unknown.
OBJECTIVES
We examined the short- and long-term effects and safety profile of bosentan in patients with PAH and complex CHD.
METHODS
We followed 14 patients with PAH and complex CHD for a mean of four years. Demographic parameters, exercise capacity assessed by the six-minute walking test (6MWT) and oxygen saturation were assessed at baseline, six months and at follow-up.
RESULTS
Mean age was 37.1 ± 11.7 years; 90% were in WHO class III or IV. The most common diagnosis was pulmonary atresia with VSD (35.7%), followed by truncus arteriosus (28.6%), patent ductus arteriosus (21.4%) and transposition of the great arteries (14.3%). After six months of treatment, six-minute walking distance (6MWD) increased from 371.9 to 428.4 m (p=0.005) and functional class was improved (p=0.005). After four years, one patient discontinued bosentan due to side effects and four patients were started on sildenafil, after a mean 38 months of bosentan treatment. Mean 6MWD for patients on bosentan monotherapy (n=8) was 440.1 ± 103.8 m, whereas for patients on bosentan-sildenafil combination therapy (n=4) it was 428.8 ± 96.9 m, after four years of therapy. Two patients died during follow-up.
CONCLUSIONS
Bosentan was safe and was associated with improved exercise capacity in patients with PAH and complex CHD. This improvement was sustained for up to four years and the safety profile was similar to simple CHD patients.
Topics: Adult; Antihypertensive Agents; Bosentan; Familial Primary Pulmonary Hypertension; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Male; Prospective Studies; Sulfonamides; Time Factors
PubMed: 23351920
DOI: 10.1016/j.repc.2012.02.023 -
International Heart Journal 2015The operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood... (Review)
Review
The operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood flow. Although some recommendations exist regarding operative indications according to pathological, hemodynamic, and epidemiological factors, the evidence underlying these recommendations is not conclusive. Recently, oral pulmonary vasodilator therapy has been reported to improve outcomes in patients with idiopathic pulmonary arterial hypertension, and this therapy also appears to be effective in patients with congenital heart disease and pulmonary arterial hypertension, including those with postoperative pulmonary hypertension and Eisenmenger syndrome. It is expected that the availability of novel pulmonary vasodilator therapy will expand the operative indications in patients with congenital heart disease with left to right shunt, but there is currently insufficient evidence to definitively determine this. A multicenter double-blind study should be conducted to further examine this issue.
Topics: Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Treatment Outcome; Vasodilator Agents
PubMed: 25787792
DOI: 10.1536/ihj.14-396 -
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 -
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 -
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 -
Revista Espanola de Cardiologia May 2005Pulmonary arterial hypertension is a chronic, persistent elevation in pulmonary artery pressure without evidence of left heart failure. Pulmonary hypertension is common... (Review)
Review
Pulmonary arterial hypertension is a chronic, persistent elevation in pulmonary artery pressure without evidence of left heart failure. Pulmonary hypertension is common in patients with adult congenital heart disease and is usually the result of an increase of pulmonary blood flow through a large left to right shunt. This condition is progressive and patients are symptomatic and usually die between the third and fifth decades of life. To date, there is no standardized treatment for this condition and a general policy of non-intervention to avoid destabilization of the balanced physiology is recommended. Intravenous prostanoids have been shown to have an effect but they are invasive and associated with major side effects. Lung and combined heart and lung transplantation might be a therapeutic option for selected patients. However, donor shortage is a major issue. Oral advanced therapies have been recently shown to improve haemodynamics and survival in idiopathic pulmonary hypertension or in pulmonary hypertension related to scleroderma and may have a role in patients with pulmonary hypertension secondary to congenital heart disease.
Topics: Eisenmenger Complex; Heart Diseases; Humans; Hypertension, Pulmonary
PubMed: 15899189
DOI: No ID Found