Cardiovascular genomics: implications for acute and critical care nurses.

As genomic health care becomes commonplace, nurses will be asked to provide genomic care in all health care settings including acute care and critical care. Three common cardiac conditions are reviewed, Marfan syndrome, bicuspid aortic valve, and hypertrophic cardiomyopathy, to provide acute care and critical care nurses with an overview of these pathologies through the lens of genomics and relevant case studies. This information will help critical care nursing leaders become familiar with genetics related to common cardiac conditions and prepare acute care and critical care nurses for a new phase in patient diagnostics, with greater emphasis on early diagnosis and recognition of conditions before sudden cardiac death.

Marfan syndrome teaching algorithm: does it make a difference?

Marfan Syndrome (MFS) is an autosomal dominant, connective tissue disorder that is due to a deficiency in the structural protein, fibrillin. MFS patients are more likely to experience aortic aneurysms and dissections, dislocated lens, and/or severe musculoskeletal deformities than non-MFS patients. Attainment of a longer lifespan in MFS patients is directly dependent on vigilant blood pressure (BP) control, frequent cardiology surveillance, annual eye exams and frequent dental hygiene visits. This study evaluated the effect of a Marfan Syndrome Teaching algorithm (MFSTA) on 20 MFS patients, with regard to BP management, cardiovascular medication adherence; adherence to activity restrictions; and attendance at scheduled eye, cardiology and dental exams. This study demonstrated adherence improvement in the attendance at scheduled cardiology, ophthalmology, and dental exams from 50%, 55% and 70% prior to the study, respectively, to 95%, 90% and 100% post study. Furthermore, subject adherence with self-administration of ordered cardiovascular medications increased from 50% (pre-study) to 93.3% (93.3%), and subject adherence with activity restrictions escalated from 70% (pre-study) to 95% (post study). All subjects demonstrated proficiency in regular testing and recording of their blood pressure. There was no significant change in the mean systolic BP (SBP) for 13 of the subjects who had both pre- and post-intervention BP recording, although the post intervention SBP was slightly higher (p = 0.30). However all subjects in the intervention period demonstrated a mean SBP of 124.7 mm Hg, with standard deviation (SD) of 12.9 mm Hg. Limited pre-intervention BP readings of 7 subjects prevented a pre- and post-SBP comparison. The MFSTA model should be considered for other patient populations involving chronic cardiovascular healthcare conditions.

Processo de enfermagem aplicado a um paciente com síndrome de marfan associado a aneurisma aórtico / Proceso de enfermería aplicada a um paciente con el síndrome de marfan asociado a la aneurisma aórtica / Nursing process applied to a patient with marfan syndrome associated with aortic aneurysm

Trata-se de um caso clínico que teve como objetivo traçar diagnósticos, intervenções e resultados de enfermagem em um paciente com Síndrome de Marfan internado na unidade de terapia intensiva no pós-operatório de correção de aneurisma de aorta. Foi desenvolvido em um Hospital Universitário, localizado no município de Natal-Brasil, em abril de 2011. Entre os principais diagnósticos de enfermagem identificados, destaca-se: Débito Cardíaco Diminuído; Risco de Infecção; Dor Aguda; Risco de Glicemia Instável; Integridade da Pele Prejudicada e Ansiedade. Percebeu-se que a aplicação do processo de enfermagem neste paciente contribuiu para delimitar o campo de atuação específico da enfermagem, bem como identificar os cuidados prioritários, contribuindo para uma melhoria na qualidade da assistência (AU)

Se trata del caso clínico que tuvo como objetivo describir diagnósticos, intervenciones y resultados de enfermería en un paciente con el Síndrome de Marfan internado en la unidad de terapia intensiva en el posoperatório de corrección de aneurisma de aorta. Fue desarrollado en el hospital Universitário, localizado en el municipio de Natal-Brasil, en abril de 2011. Entre los principales diagnósticos de enfermería identificados, se destacan: Débito Cardíaco Disminuido; Riesgo de Infección; Dolor Aguda; Riesgo de Glucemia Inestable; Integridad de la Piel Afectada y Ansiedad. Se percibió que la aplicación del proceso de enfermería en este paciente contribuyó para delimitar el campo de actuación específico de la enfermería, así como identificar los cuidados prioritarios, contribyendo para una mejoria en la calidad de la asistencia (AU)

It is a clinical case which aimed to trace diagnostics, interventions and results in a patient with Marfan Syndrome hospitalized in the intensive care unit after surgery for aneurysm of the aorta. This study was developed in a University Hospital located in Natal- Brazil, in April 2011. Among the main nursing diagnoses identified, there are: Decreased Cardiac Output; Risk of Infection; Acute Pain; Risk of Unstable Blood Glucose, Impaired Skin Integrity and Anxiety. It was noticed that the application of the nursing process in this patient helped to define the specific field of the nursing work as well as to identify the main care, contributing to an improvement in quality of support (AU)

Marfan syndrome: a review.

Cardiac complications of Marfan syndrome can be fatal. This article reviews the assessment of a patient with this hereditary disorder, complications and their treatment, and nursing care.

Marfan's syndrome in pregnancy: implications for advanced practice nurses.

Marfan's syndrome is a genetic disorder that often affects the cardiovascular, pulmonary, ocular, and musculoskeletal systems. When a woman with Marfan's syndrome becomes pregnant, the hormonal stresses and changes to the cardiovascular system can put the mother at risk for serious complications. Advanced practice nurses need to understand the maternal changes associated with Marfan's syndrome as well as the genetic factors involved in order to provide holistic care.

Marfan's syndrome and surgical repair of ascending aortic aneurysms.

Marfan's syndrome is an inherited, degenerative connective tissue disorder that affects many body systems (eg, skeletal, ocular, cardiovascular, cutaneous, pulmonary, abdominal, neurologic). The cause of Marfan's syndrome is unknown, but recent genetic studies have linked this disorder to chromosome 15q15-q21.3. The characteristics associated with Marfan's syndrome require a multidisciplinary approach to patient care. This article discusses one serious complication of Marfan's syndrome-aortic root dilatation- and composite graft repairs of ascending aortic aneurysms. Physicians and nurses must be more aware of Marfan's syndrome so that life-threatening medical conditions can be evaluated and followed by health care providers.

Caring for a Marfan patient with cardiovascular complications.

The Marfan syndrome is a heritable disorder of connective tissue associated with characteristic abnormalities of the skeletal, ocular and cardiovascular systems. Common cardiovascular manifestations of this syndrome are mitral valve prolapse with mitral regurgitation and dilatation of the ascending aorta resulting in aortic insufficiency, dissection, aneurysm and/or rupture. Although the prognosis for a patient with the Marfan syndrome is significantly more favorable than it was ten years ago, the cardiovascular complications continue to greatly reduce life expectancy. This article presents an overview of the Marfan syndrome including: history and epidemiology, clinical manifestations, diagnostic criteria, surgical intervention and follow-up. A case study is outlined which focuses on priority nursing diagnoses and a plan of care.

Marfan's syndrome.

Although Marfan's syndrome is not a frequent occurrence in critical care, serious cardiovascular problems can exist, requiring intensive care. This article has identified the manifestations in those coping with Marfan's syndrome. Critical care nurses need to be aware of the clinical presentation and pathophysiology of the problem to understand the treatment and assist in patient education.

Marfan's syndrome. A personal perspective.

Marfan's patients share the same problems as anyone with a lifelong, progressive, hereditary condition. The more knowledgeable an individual is about his disease and the more willing he is to accept the responsibility for his own well-being, the more control he has over his life. Medical and life insurance is not a luxury, but an absolute must. The need for stable insurance coverage impacts career decisions. Medication is a lifelong necessity; the patient must know what medications he takes, for what purpose, and the side effects. For patients with a cardiac prosthesis, most physicians recommend a regimen of prophylactic antibiotics before dental work or any invasive procedure. It is the responsibility of the patient to do this. Craig has cancelled dental appointments because he has forgotten to take antibiotics. Craig also takes an anticoagulant because of his prosthetic valve and fears being in a major accident and bleeding to death. Because of human body contains large amounts of connective tissue, a Marfan's patient will continue to develop problems; hernias, eye problems, joint problems, and further cardiovascular complications are all possibilities. Being realistic and maintaining a positive attitude are important in effective planning and dealing with continued adversity.