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Heart Failure in Adult Congenital Heart Disease
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Andrew Constantine, Ana Barradas-Pires, Isma Rafiq, Justyna Rybicka, Michael A. Gatzoulis, Konstantinos Dimopoulos
Despite the apparent advantages of the Eisenmenger phenotype compared to other forms of PAH, adult Eisenmenger patients are significantly impaired and have a reduced survival compared with other groups of CHD patients,6 with a 10-year survival of 69% in treatment-naïve patients.66 The transition from a compensated state to progressive RV failure usually occurs in the third or fourth decade. HF is a leading cause of death in this population and has become more common in recent decades.67 There are numerous published markers of an increased mortality in Eisenmenger syndrome, which have been summarized in the multicenter study by Kempny et al. 68
Cardiac diseases in pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Saravanan Kuppuswamy, Sudarshan Balla
Pregnancy is usually very well tolerated in women with a VSD (33). Morbidity and mortality is related to the presence of pulmonary hypertension, with a higher complication rate in those patients with Eisenmenger’s physiology (31). Management of Eisenmenger syndrome is discussed below.
Cardiology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
Ostium secundum defects are more common and are high in the atrial septum; septum primum defects are lower and typically affect the AV node leading to conduction problems. Clinical features: in adults symptoms relate to onset of atrial fibrillation and heart failure; may rarely present with CVA (paradoxical embolus). An ESM is heard over the pulmonary area and there is wide, fixed splitting of the second heart sound. ECG reveals RBBB with right axis deviation (secundum) or left axis deviation (primum). Management: involves closure of the defect. Complications include: Eisenmenger’s syndrome.
Pregnancy in women with congenital heart disease: a focus on management and preventing the risk of complications
Published in Expert Review of Cardiovascular Therapy, 2023
Gurleen Wander, Johanna A. van der Zande, Roshni R Patel, Mark R Johnson, Jolien Roos-Hesselink
Management. Although pregnancy is generally not advised, when it does occur, intensive monitoring is required with serial echocardiograms and frequent MDT review. Bed rest may be advised in severe symptomatic cases. VTE prophylaxis should be prescribed, and HF treated with medications including diuretics. Oral sildenafil is safe in pregnancy. Eisenmenger’s syndrome presents more challenges during pregnancy due to cyanosis and the potential for paradoxical embolism. Women with oxygen saturation of less than 85% have poor fetal outcomes. The cyanosis increases during pregnancy due to decrease in SVR and consequent increased right to left shunt [4,62]. Maternal mortality is estimated to be around 20–50% in these women and termination should be discussed early in the pregnancy. A termination of pregnancy is high risk and should be carried out in specialized centers with dedicated cardiologists, obstetricians, and obstetric anesthetists. Care should be taken in the post-partum period when most complications occur [15].
Management of pulmonary arterial hypertension during pregnancy
Published in Expert Review of Respiratory Medicine, 2023
Kaushiga Krishnathasan, Andrew Constantine, Isma Rafiq, Ana Barradas Pires, Hannah Douglas, Laura C Price, Konstantinos Dimopoulos
The risk during pregnancy in patients with Eisenmenger syndrome remains prohibitive, even in the current era. Even though mortality is not as high as in previous decades, it is still significant, and major complications are common [39]. A recent case series of 12 pregnancies in women with Eisenmenger syndrome from India reported that one-half developed pre-eclampsia and 27% died. However, most were diagnosed with Eisenmenger syndrome during pregnancy and were treated with sildenafil monotherapy [42]. These findings highlight the risk of pregnancy in this cohort and support current guidance that advises against pregnancy. Early diagnosis of PAH and adequate pre-conception counseling can avoid pregnancy and the risk associated with termination. This may be more difficult to achieve in countries with limited resources, PAH related to unrepaired CHD is more prevalent in low- and middle-income countries. Maternal outcomes in Eisenmenger syndrome are worse in low-income countries compared to high-income countries, this may be due to late presentation and detection but reports are limited [42–45].
Updates in the management of congenital heart disease in adult patients
Published in Expert Review of Cardiovascular Therapy, 2022
Danielle Massarella, Rafael Alonso-Gonzalez
Population data estimate a lifetime prevalence of cardiac shunt lesions of 9.19 per 1000 children and 1.60 per 1000 adults. Most commonly, these are simple lesions such as atrial and ventricular septal defects and patent ductus arteriosus [114]. Natural history studies have taught us that the development of pulmonary arterial hypertension results inevitably from a longstanding or unrepaired hemodynamically significant shunt, and its prevention remains one of the foremost goals of the treatment of this class of congenital cardiac anomalies. Luckily, successive surgical eras have seen a decrease in the incidence of unrepaired lesions diagnosed in adulthood, particularly in developed countries. Nevertheless, the high morbidity and mortality associated with Eisenmenger syndrome persists today [115]. Depending on age at repair, long-term benefits to closure must be weighed carefully against procedural risks as well as those related to underlying pulmonary vascular disease. In the current era, procedural morbidity and mortality related to surgical or transcatheter approaches is exceedingly low [10].