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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 -
The Australian & New Zealand Journal of... Feb 2023Pulmonary arterial hypertension (PAH) is a high-risk condition during pregnancy, with recent literature describing mortality rates of up to 23%.
BACKGROUND
Pulmonary arterial hypertension (PAH) is a high-risk condition during pregnancy, with recent literature describing mortality rates of up to 23%.
AIM
To describe the course and outcomes of pregnancy for women with PAH in a major Australian metropolitan referral centre over a 15-year period.
METHODS
Retrospective review of medical records of all pregnant women with PAH over the period 2005-2020.
RESULTS
We report the outcomes of nine pregnancies in six women. In five women, seven pregnancies proceeded to term with birth of a healthy neonate, five vaginal births and two caesareans. Two women opted for a termination of pregnancy in the first trimester following counselling. The planning of care and patient-centred decision-making was individually tailored by a multidisciplinary team. The pulmonary hypertension clinic provided specialist support including the management of pulmonary vasodilators. All women who delivered a live offspring received neuraxial anaesthesia.
CONCLUSIONS
Women with this condition are ideally managed in a centre with expertise in PAH; counselling regarding the risks is imperative. Regional anaesthesia, irrespective of the mode of delivery, facilitated safe delivery and improved patient experience. The option of aiming for a term vaginal birth needs to be considered in these complex women.
Topics: Infant, Newborn; Pregnancy; Female; Humans; Pulmonary Arterial Hypertension; Cesarean Section; Australia; Hypertension, Pulmonary; Pregnancy Complications, Cardiovascular; Parturition; Pregnancy Outcome
PubMed: 35699259
DOI: 10.1111/ajo.13557 -
BMJ Case Reports May 2022Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging...
Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging condition to manage, further compounded if the patient is critically ill and acutely decompensated. We share our experience of managing a critically ill adult patient with ES who presented with acute decompensation due to sepsis.
Topics: Adult; Critical Illness; Eisenmenger Complex; Familial Primary Pulmonary Hypertension; Heart Defects, Congenital; Humans; Pulmonary Arterial Hypertension
PubMed: 35580953
DOI: 10.1136/bcr-2021-245549 -
Frontiers in Cardiovascular Medicine 2022Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether...
BACKGROUND
Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD.
METHODS
A retrospective cohort study was performed on 427 patients with ASD (median age at diagnosis 38 years, IQR 18-56) out of which 186 patients (44%) manifested PH. ASD closure in patients with PH was only considered in patients without Eisenmenger syndrome with pulmonary vascular resistance < 5 WU. Median follow-up duration was 18 years (IQR 9-31 years). Kaplan-Meier and Cox proportional hazards survival analyses were performed to evaluate 12 potential predictors of survival.
RESULTS
Defect closure was associated with improved long-term survival in ASD patients both with ( < 0.001) and without PH ( = 0.01) and this association was present also in patients over 40 years. The 20-year survival since diagnosis was significantly higher in patients with PH and closed ASD compared to those with PH and open ASD (65% vs. 41%). ASD closure was a significant independent predictor of long-term survival ( = 0.003) after accounting for age at diagnosis, PH, NYHA class, Eisenmenger syndrome, and mitral regurgitation. Significant negative independent predictors of survival were older age at diagnosis ( < 0.001), Eisenmenger syndrome ( < 0.001), and PH ( = 0.03).
CONCLUSION
ASD closure appears to be associated with improved long-term survival independently of age, PH, and other clinical variables.
PubMed: 35571174
DOI: 10.3389/fcvm.2022.867012 -
Pediatric Cardiology Dec 2022Eisenmenger syndrome is a life-threatening complication of congenital heart defects (CHD). Since Eisenmenger syndrome among children of repaired CHD is rare, very few...
Eisenmenger syndrome is a life-threatening complication of congenital heart defects (CHD). Since Eisenmenger syndrome among children of repaired CHD is rare, very few studies have had the necessary data to investigate its distribution in children. The current study used data collected in rural China to investigate the prevalence of Eisenmenger syndrome in children with unrepaired CHD. Data were from the 2006 to 2016 patient medical records of China California Heart Watch, which is a traveling cardiology clinic in Yunnan Province, China. Patients were included if they (1) aged 18 or below, (2) had CHD(s), and (3) the defect was not repaired by the time of the clinic visit. The prevalence of Eisenmenger syndrome was calculated in each age and defect group. Using logistic regression models, we tested whether oxygen saturation, Down syndrome, sex, and age were significantly associated with Eisenmenger syndrome. Of the 1301 study participants, ventricular septum defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA) were the most common CHD. About one-sixth of the patients had pulmonary hypertension and 1.5% had Eisenmenger syndrome. The percentages of Eisenmenger syndrome were 1.8% in VSD patients, 0 in ASD patients, and 0.9% in PDA patients. Patients in the age group between 15 and 18 years had the highest percentages of Eisenmenger syndrome (11.5%). Age and presence of Down syndrome were significantly associated with the presence of Eisenmenger syndrome. Our finding highlights the importance of early detection and correction of CHD.
Topics: Child; Humans; Eisenmenger Complex; Down Syndrome; China; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Heart Septal Defects, Atrial; Ductus Arteriosus, Patent
PubMed: 35522268
DOI: 10.1007/s00246-022-02922-z -
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 -
Pathology International Jun 2022
Topics: Adrenal Gland Neoplasms; Autopsy; Eisenmenger Complex; Humans; Paraganglioma; Pheochromocytoma
PubMed: 35394097
DOI: 10.1111/pin.13225 -
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 -
The American Journal of Case Reports Mar 2022BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal...
BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. CASE REPORT We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. In July 2020, the patient began to report neurological symptoms in the form of lower-limb numbness and weakness of lower-limb strength, with additional increasing lower-limb edema. A CT scan of the abdomen and pelvis revealed a 63×102×103 mm soft-tissue mass in the pelvis, located behind the urinary bladder. Due to the suspicion of proliferative disease, mainly of the lymphatic system, a diagnostic trephine biopsy was performed first, but no tumor cell infiltration was found. Then, the patient was qualified for diagnostic surgery. During the operation, tumor sections were harvested. Histopathological examination of the tissue sections showed extramedullary hematopoiesis in the tumor lining. Hydroxycarbamide was used as first-line treatment. However, it was not effective in controlling clinical symptoms. Therefore, the patient was qualified for radiotherapy as a second-line palliative treatment. CONCLUSIONS This report presents a patient with cyanotic heart disease and extramedullary and non-hepatosplenic hematopoiesis presenting as masses that mimicked malignancy. In this case, palliative radiotherapy effectively reduced the symptoms due to the size of the mass lesion.
Topics: Adult; Eisenmenger Complex; Hematologic Diseases; Hematopoiesis, Extramedullary; Humans; Liver; Male; Tomography, X-Ray Computed
PubMed: 35332114
DOI: 10.12659/AJCR.935141 -
Journal of the American College of... Mar 2022Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease,... (Review)
Review
Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.
Topics: Child; Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension
PubMed: 35331414
DOI: 10.1016/j.jacc.2022.01.022