Factors influencing prognosis in patients with marfan syndrome after aortic surgery

Linggen Gao1, Xianliang Zhou, Lin Zhang

  • 1Department of Cardiology, FuWai Hospital and Cardiovascular Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Abstract

Insights

For Marfan syndrome (MFS) patients undergoing aortic surgery, high blood pressure predicts death, while body mass index and smoking predict reoperation. Managing these factors may improve long-term outcomes for MFS patients.

Area of Science:

  • Cardiovascular Surgery
  • Genetics
  • Thoracic Surgery

Background:

  • Marfan syndrome (MFS) can lead to fatal aortic aneurysm, rupture, or dissection in young adults.
  • While aortic surgery improves prognosis, long-term reoperation rates remain high.
  • Factors influencing long-term outcomes in surgically treated Chinese MFS patients are not well understood.

Purpose of the Study:

  • To investigate factors influencing long-term prognosis, including reoperation and mortality rates, in Chinese patients with Marfan syndrome after aortic surgery.

Main Methods:

  • Retrospective clinical investigation at an academic medical center.
  • Included 125 Marfan syndrome patients who underwent aortic surgery.
  • Data analyzed for in-hospital/30-day mortality, survival rates, and reoperation rates.

Main Results:

  • The most common procedure was the Bentall (92 patients). In-hospital/30-day mortality was 1.6%.
  • Survival rates were 97.5% (1 year), 91.4% (5 years), and 74.2% (10 years).
  • Reoperation rates were 2.5% (1 year), 12.9% (5 years), and 32.9% (10 years). Increased systolic blood pressure predicted death; body mass index and smoking predicted reoperation.

Conclusions:

  • Identified key factors influencing prognosis in Chinese MFS patients post-aortic surgery.
  • Managing elevated systolic blood pressure, body mass index, and smoking may improve outcomes.
  • These findings aid healthcare professionals in optimizing MFS patient care after aortic procedures.

Related Concept Videos

Aneurysm IV: Nursing Management01:22

Aneurysm IV: Nursing Management

Vigilant monitoring for aneurysm rupture is essential for patients undergoing aortic surgery.Preoperative Nursing ManagementContinuously monitor the patient for manifestations of aneurysm rupture, such as pallor, weakness, tachycardia, hypotension, abdominal, back, groin, or periumbilical pain, changes in consciousness, and a pulsating abdominal mass. Regularly assess the patient's peripheral pulses.Instruct the patient to consume a clear liquid diet the day before surgery and administer...
Aortic Regurgitation III: Medical Management01:25

Aortic Regurgitation III: Medical Management

Aortic regurgitation (AR) is when the aortic valve does not close or seal properly, leading to backward blood circulation from the aorta into the left ventricle during diastole. Common causes of AR include rheumatic heart disease, congenital valve defects, and aortic root dilation. Managing AR requires a multifaceted approach to alleviate symptoms, preserve left ventricular function, and address the underlying cause of the regurgitation. Patients with symptomatic AR or significant left...
Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
Aneurysm III: Interprofessional Care01:26

Aneurysm III: Interprofessional Care

Aneurysm management involves either conservative medical therapy or surgical intervention, depending on the size and symptoms of the aneurysm. Conservative management is generally reserved for smaller, asymptomatic aneurysms, while larger or symptomatic aneurysms often necessitate surgical repair.Conservative Medical TherapyFor small, asymptomatic aneurysms, particularly abdominal aortic aneurysms (AAA) less than 5.5 centimeters in diameter, conservative medical therapy is recommended. This...
Aortic Regurgitation I: Introduction01:15

Aortic Regurgitation I: Introduction

IntroductionAortic regurgitation is characterized by the backward flow of blood from the aorta into the left ventricle during diastole and arises from the improper closure of the aortic valve. This condition results in left ventricular volume overload and can stem from both acute and chronic etiologies, each contributing uniquely to the disease's progression and symptomatology.Acute and Chronic CausesAcute aortic regurgitation often results from events that suddenly impair the integrity of the...
Cardiomyopathy VII: Pre and Post Operative Nursing Management01:28

Cardiomyopathy VII: Pre and Post Operative Nursing Management

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...