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Rheumatic mitral valve disease is a progressive heart condition in which rheumatic fever experienced in the past permanently damages the structure of the mitral valve located between the left atrium and left ventricle of the heart, causing the valve to narrow or allowing blood to leak backward. Damage developing in the valve leaflets causes thickening and calcification over time, preventing the heart from pumping blood properly. This condition, which may progress silently, disrupts the blood pressure balance of the circulatory system and leads to clinical consequences ranging from shortness of breath to severe heart failure. This structural deterioration, which directly affects quality of life, is a vital health problem that must be managed with regular cardiological follow-up and timely application of appropriate treatment strategies.
| Definition of the Disease | Rheumatic mitral valve disease is a chronic heart valve disease that generally develops after acute rheumatic fever and disrupts the structure of the mitral valve, causing narrowing (mitral stenosis), leakage (mitral regurgitation), or both to occur together. |
| Main Cause | The most common cause is acute rheumatic fever that develops following a group A beta-hemolytic streptococcal infection. The inflammatory response created by the immune system causes permanent damage to the mitral valve over time. |
| Risk Factors | Untreated throat infections during childhood, a history of acute rheumatic fever, low socioeconomic conditions, crowded living environments, and limited access to healthcare services create risk. |
| Affected Valve | The mitral valve is affected most frequently. In some patients, the aortic valve may also be involved at the same time. |
| Development of the Disease | Thickening, calcification, reduced movement of the leaflets, and hardening of the subvalvular structures develop. These changes obstruct blood flow or cause backward leakage. |
| Symptoms | Shortness of breath with exertion, easy fatigue, palpitations, chest discomfort, cough, bloody sputum, swelling in the feet, and shortness of breath at rest in advanced stages may occur. |
| Early-Stage Findings | The disease may remain asymptomatic for many years. The first symptoms are usually shortness of breath during exercise and reduced exercise capacity. |
| Advanced-Stage Findings | Symptoms of heart failure, atrial fibrillation, pulmonary hypertension, severe shortness of breath, and exercise intolerance may develop. |
| Physical Examination Findings | A heart murmur, irregular heart rhythm, an opening snap, findings related to fluid accumulation in the lungs, and edema in advanced cases may be detected. |
| Diagnostic Methods | Echocardiography is the main diagnostic method. Electrocardiography (ECG), chest X-ray, transesophageal echocardiography, cardiac catheterization, and CT or MRI when necessary may also be used. |
| Importance of Echocardiography | It is the most important examination for evaluating valve area, valve movements, the degree of narrowing or leakage, the size of the heart chambers, and pulmonary artery pressure. |
| Problems Seen in Mitral Stenosis | Increased pressure in the left atrium, elevated pressure in the pulmonary vessels, shortness of breath, and a risk of clot formation may occur. |
| Problems Seen in Mitral Regurgitation | Because blood leaks back into the left atrium, the heart may enlarge over time and heart failure may develop. |
| Possible Complications | Atrial fibrillation, stroke, heart failure, pulmonary hypertension, infective endocarditis, and, rarely, sudden clinical deterioration may occur. |
| Treatment Goals | The goals are to reduce symptoms, prevent complications, preserve heart function, and perform valve intervention or surgery at the appropriate time when necessary. |
| Medication Treatment | Diuretics, medications that control heart rate, rhythm-regulating drugs, anticoagulants when necessary, and long-term penicillin prophylaxis to prevent recurrence of rheumatic fever in suitable patients may be used. |
| Interventional Treatment | Percutaneous balloon mitral valvuloplasty may be performed in severe mitral stenosis with suitable anatomy. |
| Surgical Treatment | Valve repair or mitral valve replacement is preferred in patients for whom interventional treatment is unsuitable or unsuccessful. |
| Conditions That May Require Surgery | Marked symptoms caused by severe valve disease, progressive heart failure, severe valve stenosis or regurgitation, and deterioration in heart function may require surgery. |
| Follow-Up Process | Patients should be monitored with regular cardiology examinations and echocardiography at specified intervals. The frequency of follow-up is determined according to the severity of the disease. |
| Lifestyle Recommendations | Controlling salt intake, planning regular physical activity according to the physician’s recommendation, quitting smoking, maintaining an ideal weight, and protection from infections are recommended. |
| Regarding Pregnancy | Severe mitral valve disease may pose risks for both the mother and baby during pregnancy. It is important for patients planning pregnancy to undergo a cardiology evaluation beforehand. |
| Prevention | Preventing acute rheumatic fever through early and appropriate antibiotic treatment of streptococcal throat infections is the most effective preventive method. Secondary preventive antibiotic treatment is important in patients who have had rheumatic fever. |
| Conditions Requiring Emergency Medical Evaluation | Emergency medical care should be sought in the event of sudden severe shortness of breath, fainting, chest pain, rapid or irregular heartbeat, signs of stroke, or severe swelling in the legs. |
| Prognosis | The course of the disease depends on the degree of valve damage, response to treatment, and the complications that develop. With regular follow-up and appropriate treatment, many patients can maintain a good quality of life for many years. |
What Is Rheumatic Mitral Valve Disease?
In a healthy individual, the area of the mitral valve is approximately 4 to 6 cm². This width allows oxygenated blood to pass into the heart’s main pumping chamber without encountering any resistance. However, when the rheumatic process begins, the thin and flexible leaflets of the valve gradually start to thicken. The corners where the valve leaflets meet gradually adhere to one another. In addition, the parachute-cord-like supporting tissues that hold the valve from below are also affected by this inflammatory process and become shortened, thickened, and less flexible.
All these structural deteriorations restrict the movement of the valve as a whole. On ultrasound images, instead of opening freely, the valve develops a domed, hockey-stick appearance as though it is being forced. This narrowing process begins to alter the working dynamics of the heart when the valve area falls below 2 cm². In some cases, the contraction of the valve tissue becomes so pronounced that the valve cannot close, and some of the blood leaks backward with each heartbeat, meaning in the direction from which it came. This condition is a progressive process that makes the mechanical functioning of the heart more difficult, disrupts internal pressure balances, and gradually strains the structure of the heart muscle.
How Does Rheumatic Mitral Valve Disease Affect the Other Organs of the Body?
As the area of the mitral valve narrows, blood in the upper left chamber of the heart has difficulty passing into the lower chamber. Just as water accumulates in a sink with a blocked drain, blood begins to pool in this upper chamber. This increases the pressure inside and gradually causes the left atrium of the heart to stretch and enlarge like a balloon. Unfortunately, this increase in intracardiac pressure is not a problem limited only to the heart.
Oxygenated blood in the lungs tries to flow through the vessels into this enlarged upper chamber of the heart. However, because the pressure inside is high, blood also begins to pool backward in the pulmonary vessels. This accumulation of blood in the pulmonary capillaries may cause blood pressure in the lungs to rise above 30 mmHg even at rest. This increased pressure causes fluid to leak into the lung tissue and prevents the patient from breathing comfortably.
The process does not stop there. The right side of the heart, which is forced to pump blood against the high pressure in the lungs, is not accustomed to such a heavy load. Over time, the muscles on the right side of the heart become fatigued and enlarged, and a condition called right heart failure may develop. This causes deoxygenated blood returning from the body to the heart to pool in the vessels as well, creating a foundation for much broader circulatory problems such as liver enlargement, fluid accumulation in the abdomen, and visible swelling in the ankles.
What Complaints Occur When Rheumatic Mitral Valve Disease Progresses?
In the early stages of the disease, when the valve area has not yet narrowed too much, many people feel no problems in their daily lives. The body somehow adapts to this slowly developing narrowing. However, when the valve area begins to fall below 1.5 cm², the condition changes. Symptoms that are initially felt only during strenuous activities or while walking uphill begin to appear while walking on level ground and even at rest as the disease progresses.
This decline in patients’ exercise capacity is evaluated by dividing it into specific classes on an international scale. While people in mild stages can perform their daily activities comfortably, individuals in advanced stages may struggle even when moving from one room to another at home and may wake from sleep at night with a sensation of suffocation. The addition of rhythm disorders that cause the heart to beat rapidly significantly increases the severity of the complaints.
The main complaints most frequently encountered clinically in the advanced stages of this disease are as follows:
- Shortness of breath
- Palpitations
- Easy fatigue
- Chest pain
- Bluish discoloration
- Swelling in the legs
- Waking at night
Which Methods Are Used to Diagnose Rheumatic Mitral Valve Disease?
An experienced physician can obtain important clues about the severity of the disease even while listening to and examining the patient. In advanced stages, when the heart cannot pump enough blood, the body constricts the vessels in the facial area to direct blood to the vital organs. This may lead to a distinctive facial appearance characterized by a pinkish redness on the cheeks and mild paleness or bluish discoloration of the lips. The intensity and rhythm of the heart sounds heard with a stethoscope provide highly valuable information about the level of calcification of the valve and the condition of its opening.
However, advanced imaging methods are required to measure the valve structure with millimetric precision and observe changes in the heart chambers. With ultrasound performed through the chest (echocardiography), the valve area is measured and the pressure difference created as blood passes through the valve is detected. In some cases, the patient’s complaints may not correspond with the ultrasound findings at rest; in such situations, the test is repeated while the patient exercises. In patients for whom a closed intervention is planned, confirming whether there is a clot inside the heart is a critically important step.
The main methods used during clinical evaluation and diagnosis are as follows:
- Physical examination
- Transthoracic echocardiography
- Transesophageal echocardiography
- Exercise test
- Cardiac catheterization
How Is Suitability for the Balloon Procedure Determined in Rheumatic Mitral Valve Disease?
Balloon treatment performed without opening the rib cage may not be a suitable option for every patient. When making this decision, the anatomical structure, flexibility, and amount of calcification of the valve must be analyzed in great detail. For this purpose, certain special scoring systems that are used worldwide and numerically express the condition of the valve are available.
The best known of these systems evaluates the valve from four different aspects and assigns each parameter a score between 1 and 4. If the valve can move freely, the leaflets are not excessively thickened, there is no or only minimal calcification, and the supporting ligaments that hold the valve have preserved their flexibility, the valve is considered highly suitable for the balloon procedure. A low total score is the most important indicator that the valve can be gently opened with a balloon without tearing and that the procedure is likely to be successful.
However, if the valve leaflets are excessively thickened, covered with a dense layer of calcium, and have taken on an almost stone-like appearance, the risk that the valve will fail to open properly or tear uncontrollably when the balloon is inflated increases significantly. Surgical methods are generally recommended directly for patients in this condition.
The structural parameters examined in particular during this preliminary evaluation performed with an echocardiography device are as follows:
- Valve mobility
- Valve thickness
- Amount of calcification
- Condition of the supporting tissues
- Presence of leakage
How Is the Closed Balloon Procedure Performed in the Treatment of Rheumatic Mitral Valve Disease?
The procedure performed in patients whose valve anatomy is found suitable for balloon treatment is a process based on highly precise and millimetric calculations. The procedure is usually performed while the patient is conscious and only the area where the procedure will be carried out is numbed with local anesthesia. The vein in the groin is entered with the help of a small needle, and thin, flexible tubes (catheters) are advanced toward the heart through this route.
After reaching the right side of the heart, one of the most delicate stages of the procedure begins. The thin, curtain-like wall separating the right and left atria of the heart is safely passed through with a special needle. In this way, the left side where the mitral valve is located is reached. Then, a specially designed balloon system in a deflated state is advanced over a wire and positioned precisely in the middle of the narrowed valve.
Under imaging guidance from an X-ray device (fluoroscopy), the balloon is inflated in a controlled manner. The distinctive hourglass-like design of this balloon slowly separates the adhered corners of the valve using mechanical force while attempting not to damage the tissue. The inflation process lasts a few seconds, after which the balloon is immediately deflated. The immediate success of the procedure is understood by confirming how much the valve has widened through simultaneous measurements. Once sufficient opening has been achieved, all equipment is removed from the body.
What Are the Advantages of the Balloon Method in the Treatment of Rheumatic Mitral Valve Disease?
Compared with open-heart surgery that completely replaces the valve, the balloon method provides very clear differences in patients’ quality of life and recovery period. In open surgery, the breastbone must be cut, the heart must be stopped, and the patient must be connected to a heart-lung machine. None of these are required with the balloon method; treatment is provided while the heart continues pumping blood in its natural rhythm.
In addition, patients who receive a mechanical valve through open surgery must use powerful blood-thinning medications for life, and the dosage of these medications must be monitored very closely to prevent blood from clotting on the valve. Because the valve’s own natural structure is preserved in balloon treatment, patients with a regular heart rhythm generally do not face such a lifelong requirement. The improvement in complaints after the procedure is largely satisfactory, and the resulting state of well-being may last for years.
The main advantages of closed balloon treatment performed through the groin compared with open-heart surgery are as follows:
- The rib cage is not opened
- General anesthesia is not required
- The heart is not stopped
- No surgical scar forms
- The need for intensive care is reduced
- The hospital stay is short
- The recovery process is fast
- The need for blood thinners is reduced
- It can be repeated when necessary
Why Is the Clip Method Not Preferred in Advanced Rheumatic Mitral Valve Disease?
One of the innovations in medicine involves methods that aim to reduce blood leakage by securing mitral valve leaflets that cannot close completely with a clip in the middle in patients with a high surgical risk. However, this advanced clipping method is effective in structural problems where the valve has preserved its flexibility and only has difficulty joining together. When rheumatic processes are involved, the use of this option is generally unsuitable.
Rheumatic disease thickens and calcifies the leaflets, turning them into an almost hard and inflexible layer. In addition, the corners of the valve are already adhered to each other. Attempting to grasp and pull such hard and rigid tissue together with a tiny clip makes it difficult for the clip to attach to the valve tissue. The strained tissue may tear, or the function of the valve may deteriorate further.
Moreover, the valve area is already narrowed in the rheumatic process. Placing a clip in the middle and dividing the valve into two small openings may suddenly and dangerously increase the degree of narrowing, leading the patient into a much more difficult clinical condition. For these reasons, clip-like devices are not a treatment alternative in this patient group.
Can a Closed Valve Procedure Be Performed in Patients Who Have Undergone Surgery Due to Rheumatic Mitral Valve Disease?
The situation is somewhat different for patients who underwent open-heart surgery in previous years, had the rheumatic valve removed, and received a biological tissue-based prosthetic valve in its place. Biological valves have an average lifespan of 10–15 years, and when this period ends, problems such as renewed calcification, narrowing, or backward blood leakage may occur in the valve. Because a second open-heart surgery carries major risks, a different intervention method performed through the groin called “Valve-in-Valve” may be used in these patients.
In this procedure, a new biological valve in a folded state is advanced through the groin to the heart and opened after being positioned directly inside the frame of the deteriorated biological valve. Just as a stent opens a vessel, the new valve attaches securely inside the old one and begins working immediately. In this group of patients who have undergone previous surgery, the success rate of the procedure is quite high and it provides significant recovery.
However, when this closed method is attempted directly in patients who have never undergone surgery and whose native valve is excessively calcified, the results are not very satisfactory. This is because the patient’s natural valve does not have a proper frame that can hold the newly implanted valve securely in place. The possibility that the new valve may shift or that blood may leak around its edges is quite high. Therefore, for now, this technology is mainly a suitable option for patients who previously received a biological valve and whose valve has deteriorated.
How Does the Process Progress Before and After Interventions for Rheumatic Mitral Valve Disease?
It should not be forgotten that a successful intervention is possible not only through skill in the angiography laboratory but also through a carefully planned preparation period before and after the procedure. Before the procedure, the medications used by patients, especially blood thinners, must be adjusted according to a special schedule. To prevent bleeding, these medications are discontinued a certain period in advance and replaced with shorter-acting medications that are easier to control.
Patients are given plenty of intravenous fluid support to protect the kidneys from the imaging fluids used during the procedure. Because patients with diabetes should not remain fasting for long periods, interventions are generally scheduled for the early morning and blood glucose levels are closely monitored. There are also certain stages that must be considered before discharge during the post-procedure period.
The main steps in preparation before the intervention and follow-up after the procedure are as follows:
- Performing blood tests
- Adjusting medication doses
- Ensuring the fasting period
- Intravenous fluid supplementation
- Cleaning the groin area
- Applying pressure to the groin after the procedure
- Monitoring with a rhythm device
- Regular outpatient clinic check-ups
Frequently Asked Questions
Prof. Dr. Kadriye Orta Kılıçkesmez is one of the leading figures in the field of Turkish cardiology. She was born on January 24, 1974, in Tekirdağ. After completing her undergraduate education at Istanbul University Cerrahpaşa Faculty of Medicine, she chose cardiology as her specialty and received her specialist training at the Cardiology Institute of the same university. In 2015, she was appointed by the university to establish the Şişli Etfal cardiology clinic and Angio laboratory. Becoming a professor in 2017, Kadriye Kılıçkesmez established the cardiology clinic and Angio laboratory of Prof. Dr. Cemil Taşçı Hospital in 2020 and ensured that the clinic became a training clinic.
