A review of the management of pulmonary arterial hypertension associated with congenital heart disease.

Approximately 5-10% of adolescent and adult patients with congenital heart disease (CHD) will develop pulmonary arterial hypertension (PAH). Patients with PAH associated with CHD (PAH-CHD) exhibit several similarities to those with idiopathic and other associated forms of PAH, especially with regards to their non-specific, cardinal symptoms. The development of PAH-CHD can lead to lifelong impairment although, paradoxically, survival may be better versus idiopathic PAH. Patients with PAH-CHD may experience social limitations, and emotional and psychological issues, arising from their disease burden. Nurses and other allied healthcare professionals are well placed to deliver the individually-tailored care that patients with PAH-CHD require. Activities known to be of particular benefit include patient engagement and education, patient empowerment, colleague training, and ensuring effective communication across the multidisciplinary team. Recent developments in the management of PAH-CHD have led to changes in the medical needs and optimal care of this patient population. This review aims to provide an overview of the natural course, diagnosis, symptoms and impact of PAH-CHD. We also aim to communicate the current standards in management of patients with PAH-CHD, and how their outlook can be improved in the future.

What do cardiovascular nurses know about the hematological management of patients with Eisenmenger syndrome?

AIM: We investigated the level of knowledge of hematological management of patients with Eisenmenger syndrome among general cardiovascular nurses and nurses who specialize in congenital heart disease (CHD). METHODS: We conducted a survey at two international conferences attended by cardiovascular nurses. Nurses were asked to complete a questionnaire comprising two questions and three clinical case scenarios. Overall, 89 nurses participated (response rate 90.8%), 43 of whom specialized in CHD. RESULTS: The level of knowledge displayed among cardiovascular nurses is poor. About one-third of nurses not specialized in CHD recognized the definition of Eisenmenger syndrome and knew what normal hematocrit levels are. With respect to the cases presented, less than 10% of the nurses could give a correct answer. The level of knowledge of specialized nurses was significantly higher, but also here, important gaps in the level of knowledge could be observed. Less than two-thirds knew the reference values of hematocrit and knew the appropriate management in two cases. Less than half of the specialized nurses knew about the procedure of isovolumic phlebotomy. CONCLUSION: The level of knowledge displayed by cardiovascular nurses regarding the hematological management of patients with Eisenmenger syndrome is poor. Also the knowledge of nurses specialized in CHD could be improved.

Eisenmenger syndrome: A clinical review.

Eisenmenger syndrome is a medical condition, characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Patients with Eisenmenger syndrome are not very prevalent in cardiovascular nurses' practice. However, nurses need to have some basic knowledge about the syndrome, in case they have to care for such a patient in their clinical practice. In this article, we describe the epidemiology, outcome, pathophysiology, clinical presentation, medical management and counseling issues of this condition.

Congenital intracardiac shunting in the adult: outline for nursing care.

Adults with an untreated intracardiac shunt present a unique challenge to the critical care nurse. The disease process necessitates an understanding of cardiac anatomy and physiology in order to determine the etiology of the cardiac defect and resulting shunt. An understanding of fluid and pressure dynamics is also of importance since anatomic shunting alters usual adult hemodynamics. Arterial and mixed venous oximetry monitoring is most helpful in determining the response to therapies. One must rethink some of the hemodynamic goals that are traditionally used in caring for adults with primary left ventricular dysfunction. Therapeutic response is usually limited to a narrow hemodynamic range. Many of these patients have superimposed health problems such as atherosclerosis, hypertension, and cigarette smoking. Therefore, the critical care nurse is confronted with caring for a patient with unique problems that test creativity and assessment skills.