Mahaim fibers coexisting with coarctation of aorta and bicuspid aortic valve

Mohammad H Namazi1, Morteza Safi1, Amir Momenizadeh1

  • 1Cardiovascular Research Center, Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Future Cardiology
|November 14, 2018
PubMed

Insights

A rare combination of Mahaim fibers, coarctation of the aorta, and bicuspid valve was identified in a young patient with supraventricular tachycardia. Successful treatment involved percutaneous intervention and catheter ablation, resolving symptoms.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Electrophysiology

Background:

  • Mahaim fibers are accessory pathways that can cause supraventricular tachycardia.
  • Coarctation of the aorta is a congenital narrowing of the aorta.
  • Bicuspid aortic valve is a common congenital heart defect where the aortic valve has two leaflets instead of three.

Observation:

  • A young patient presented with palpitations and supraventricular tachycardia.
  • Cardiac studies revealed a rare coexistence of Mahaim fibers, coarctation of the aorta, and a bicuspid aortic valve.
  • This specific combination of congenital cardiac abnormalities had not been previously reported.

Findings:

  • Detailed cardiac investigations confirmed the presence of all three congenital abnormalities.
  • Percutaneous intervention successfully treated the coarctation of the aorta.
  • Catheter-based ablation effectively treated the Mahaim fibers.
  • Both interventions led to the resolution of the patient's symptoms.

Implications:

  • This case highlights the importance of comprehensive cardiac evaluation in patients with complex congenital heart disease.
  • The successful combined interventional approach suggests a viable treatment strategy for similar rare presentations.
  • Further research into the genetic and developmental links between these conditions may be warranted.

Related Concept Videos

The Aorta01:14

The Aorta

The aorta is the largest artery in the human body. It originates from the left ventricle of the heart and extends down to the abdomen, where it splits into two smaller arteries. Structurally, it can be divided into four main parts: the ascending aorta, the aortic arch, the thoracic aorta, and the abdominal aorta.
The average diameter of the aorta is approximately 2-3 cm, but the size can vary depending on the section of the aorta and the individual's age, sex, and body size. The aorta is...
2.8K
Anatomy of the Heart01:27

Anatomy of the Heart

The human heart is made up of three layers of tissue that are surrounded by the pericardium, a membrane that protects and confines the heart. The outermost layer, closest to the pericardium, is the epicardium. The pericardial cavity separates the pericardium from the epicardium. Beneath the epicardium is the myocardium, the middle layer, and the endocardium, the innermost layer. There are four chambers of the heart: the right atrium, the right ventricle, the left atrium, and the left ventricle.
119.8K
The Arch of Aorta01:10

The Arch of Aorta

The coronary arteries, originating from the ascending aorta, bifurcate from two sinuses located within the ascending aorta. Positioned just above the aortic semilunar valve, these sinuses house essential aortic baroreceptors and chemoreceptors, crucial for maintaining cardiac function. The left coronary artery and the right coronary artery branch off from the left posterior and anterior aortic sinuses, respectively.
Encircling the heart, the coronary arteries form a ring-like structure before...
1.7K
Thoracic Aorta01:15

Thoracic Aorta

The thoracic section of the aorta begins at the T5 vertebra and extends to the T12 level at the diaphragm, initially progressing through the mediastinum to the left of the spinal column. Throughout its course in the thoracic segment, the thoracic aorta emits various offshoots known collectively as visceral and parietal branches. The branches that predominantly supply blood to visceral organs are termed visceral branches and include bronchial, pericardial, esophageal, and mediastinal arteries,...
1.7K
Abdominal Aorta01:25

Abdominal Aorta

Once the aorta traverses the diaphragmatic plane at the aortic hiatus, it is known as the abdominal aorta. This anatomical structure is positioned leftward of the spinal column, encased within a cocoon of adipose tissue behind the peritoneal cavity. It terminates at the L4 vertebra, where it splits into the common iliac arteries. Prior to this bifurcation, the abdominal aorta gives rise to several vital branches.
The celiac trunk, a singular artery, divides into the left gastric artery, which...
2.3K
Heart Valves01:16

Heart Valves

The human heart is a complex organ with an intricate system of valves that regulate blood flow. There are two main types of valves: atrioventricular (AV) valves and semilunar valves.
The AV valves prevent the backflow of blood from the ventricles to the atria during ventricular contraction. These valves function with the assistance of the chordae tendineae and papillary muscles. When the ventricles are relaxed, the chordae tendineae are slack, allowing blood to flow from the atria into the...
12.1K