What are FIVE non-cardiac clinical features of Marfan’s syndrome?

Correct Answer: A

Rationale: In the context of pediatric nursing and cardiovascular disorders, understanding Marfan's syndrome is crucial. Marfan's syndrome is a genetic disorder that affects the connective tissue, leading to various clinical features. Ectopia lentis, the correct answer (A), refers to the dislocation of the lens in the eye, which is a hallmark feature of Marfan's syndrome due to weakened zonular fibers. This clinical sign is pathognomonic for Marfan's syndrome. The incorrect options (B, C, D) do not represent non-cardiac clinical features of Marfan's syndrome. Pectus excavatum or carinatum, pes planus, and scoliosis are musculoskeletal manifestations commonly seen in Marfan's syndrome, but they are not specific to this condition and can be present in other disorders as well. It is crucial for nurses to recognize the unique non-cardiac clinical features of Marfan's syndrome to aid in early diagnosis and appropriate management. Educationally, understanding the distinct clinical features of genetic syndromes like Marfan's syndrome enhances the nurse's ability to provide holistic care to pediatric patients. By differentiating between specific manifestations, nurses can contribute to timely referrals to specialists, genetic counseling, and tailored interventions to improve patient outcomes. This knowledge underscores the importance of a comprehensive assessment and collaborative approach in pediatric nursing practice.

Correct Answer: C

Rationale: In Kawasaki Disease, a giant coronary artery aneurysm is defined as having both a diameter of ≥8 mm and a Z score ≥10. This combination indicates a significant dilation of the coronary artery, putting the child at higher risk for complications such as thrombosis or rupture. Option A (≥8 mm in diameter) alone is not specific enough as an aneurysm could be large in diameter but not necessarily abnormal based on the patient's body size. Option B (Z score ≥10) is a more accurate measure as it considers the patient's body surface area, making it a more reliable indicator of abnormality in coronary artery size. Educationally, understanding the criteria for defining a giant coronary artery aneurysm in Kawasaki Disease is crucial for pediatric nurses caring for these patients. It helps in early detection, appropriate monitoring, and timely intervention to prevent serious cardiac complications. Nurses need to be well-versed in the diagnostic criteria to ensure optimal care for children with Kawasaki Disease.

Correct Answer: D

Rationale: In the context of pediatric nursing and cardiovascular disorders, understanding the absolute contraindications for cardiac transplantation is crucial for providing safe and effective care to patients. The correct answer, option D) Drug abuse, is an absolute contraindication for cardiac transplantation due to the increased risk of non-compliance with post-transplant medication regimens, potential interactions between immunosuppressant drugs and substances of abuse, and the overall impact on the success of the transplant. Option A) Active infection is a relative contraindication that can be managed or treated before transplantation to minimize the risk of complications. Option B) Ongoing malignancy is also a relative contraindication that may require treatment and monitoring but does not necessarily exclude a patient from transplantation. Option C) Medical non-compliance is a serious concern, but it can be addressed through education, support, and close monitoring to improve patient adherence. Educationally, understanding these contraindications helps pediatric nurses make informed decisions, provide comprehensive care, and advocate for the best interests of their patients. By prioritizing drug abuse as an absolute contraindication, nurses can work towards optimizing transplant outcomes and ensuring the long-term well-being of pediatric patients with cardiovascular disorders.

Correct Answer: A

Rationale: In a 4-year-old girl presenting with a heart rate of 40 bpm, an atrial rate of 92 bpm, and AV-dissociation, the most likely etiology among the options provided is A) Congenital CHB from maternal Lupus. This condition occurs when maternal autoantibodies cross the placenta, leading to inflammation and scarring of the fetal AV node, resulting in heart block. Option B) Fetal myocarditis is less likely as it primarily affects the myocardium, not the conduction system. Option C) CCTGA (Congenitally Corrected Transposition of the Great Arteries) typically presents with different ECG findings. Option D) Medication-induced heart block is possible but less common in a pediatric patient without relevant medication history. Educationally, understanding the etiology of heart block in pediatric patients is crucial for nurses caring for children with cardiovascular disorders. Recognizing the association between maternal autoimmunity and congenital heart block highlights the importance of maternal health in fetal development. This knowledge can guide prenatal counseling, early detection, and appropriate management of congenital heart block in pediatric patients.

Correct Answer: B

Rationale: In pediatric nursing, understanding cardiovascular disorders, specifically left ventricular outflow tract obstruction in atrioventricular septal defects (AVSD), is crucial for providing effective care. The correct answer is B) Subaortic fibromuscular ridge. This is because a subaortic fibromuscular ridge is a common anatomical anomaly in AVSD that can cause obstruction of the left ventricular outflow tract. Option A) Gooseneck is incorrect as it does not represent a mechanism for LV outflow tract obstruction in AVSD. Option C) Septal hypertrophy is also incorrect as it does not specifically relate to the obstruction at the LV outflow tract in this context. Option D) Abnormal LAVV chordal attachments are not a known mechanism for LV outflow tract obstruction in AVSD. Educationally, understanding these mechanisms is essential for nurses caring for pediatric patients with AVSD. By knowing the correct anatomical features that can lead to obstruction, nurses can anticipate complications, provide appropriate interventions, and collaborate effectively with the healthcare team to optimize patient outcomes. This knowledge enhances the quality of care provided to pediatric patients with cardiovascular disorders.