LUTEMBACHER SYNDROME

Question 1

a rare form of congenital heart disease characterized by the combination of two heart defects: a congenital atrial septal defect (ASD) and acquired mitral stenosis.

Answer 1

Lutembacher syndrome

Question 2

Lutembacher syndrome is a rare form of congenital heart disease characterized by the combination of two heart defects:

Answer 2

Congenital atrial septal defect (ASD)
Aquired Mitral Stenosis

Question 3

a hole in the wall (septum) that divides the two upper chambers of the heart (atria), allowing blood to flow between them instead of circulating properly through the heart and to the rest of the body

Answer 3

Atrial Septal Defect

Question 4

on the other hand, refers to the narrowing of the mitral valve, which is located between the left atrium and left ventricle. This narrowing impedes blood flow from the left atrium to the left ventricle.

Answer 4

Mitral Stenosis
(Acquired)

Question 5

Types There are 4 types of Atrial Septal Defects:

Answer 5

1. Ostium Secundum Atrial Septal Defect (OS-ASD) (occurs in the middle part of atrial septum)
2. Ostium Primum Atrial Septal Defect (OP-ASD): (located at the lower part of the atrial septum, near the atrioventricular (AV) valves)
3. Sinus Venosus Atrial Septal Defect: (It occurs near the junction of the superior or inferior vena cava with the right atrium).
4. Coronary Sinus Atrial Septal Defect: It involves the part of the atrial septum that separates the coronary sinus (which returns venous blood from the heart muscle to the right atrium) from the left atrium.

Question 6

Mitral stenosis can be classified by: Etiology

Answer 6

1. Rheumatic Mitral Stenosis
2. Congenital Mitral Stenosis
3. Calcific Mitral Stenosis

Question 7

The most common cause worldwide. It results from rheumatic fever, an autoimmune reaction to a streptococcal infection, leading to scarring and deformation of the mitral valve.

Answer 7

Rheumatic Mitral Stenosis

Question 8

Present from birth, this type involves abnormalities in the development of the mitral valve.

Answer 8

Congenital Mitral Stenosis

Question 9

Often seen in the elderly, this type is due to the calcification of the mitral valve apparatus.

Answer 9

Calcific Mitral Stenosis

Question 10

MVA >1.5 cm².

Answer 10

Mild Mitral Stenosis

Question 11

MVA 1.0-1.5 cm².

Answer 11

Moderate Mitral Stenosis

Question 12

MVA <1.0 cm².

Answer 12

Severe Mitral Stenosis

Question 13

The signs and symptoms of Lutembacher’s syndrome in a patient are presented:

Answer 13

● Palpitations or irregular heartbeat

● Right ventricular overload and right- sided heart failure

● Pulmonary congestion

● Loud mitral S1 and wide fixed split of pulmonary S2

● III/IV mid diastolic murmur, early systolic murmur

● Dyspnea (shortness of breath), especially during physical exertion or when lying flat)

● Fatigue and weakness

● Edema (swelling), particularly in the legs and abdomen

● Chest pain or discomfort

● Cyanosis (bluish discoloration of the skin) in severe cases

Question 14

Management
Surgical intervention: There are several types of surgeries depending on the cause of Lutembacher’s Syndrome (ASD Primum or ASD Ostium Secundum with Mitral Stenosis)

Answer 14

a. Percutaneous transcatheter therapy
b. Transcatheter therapy of balloon valvuloplasty to correct MS
c. Device closure of ASD
d. Reconstructing of the mitral and tricuspid valve

Question 15

Medications: Diuretics may be prescribed to reduce fluid overload and pulmonary congestion. Additionally, medications such as ___________ or __________ may be used to control heart rate and blood pressure.

Answer 15

Beta-blockers / Calcium channel-blockers