Double Whammy Cases of Severe Mitral Stenosis in Peripartum: A Survival Case Series

Background: Valvular heart disease in pregnancy is still not widely studied. The combination of mitral stenosis and the physiology of pregnancy for both mother and fetus often result in poor hemodynamics, and management during labor and peripartum period greatly determines the prognosis of both lives. Case: A 42 years old G3P2A0 (Case A) and A 33 years old G3P1A1 (Case B) both had a history of previous SC labor, presented worsening shortness of breath since 2 nd trimester, coughing and swelling in both legs, also unable to rest in a flat position. especially, case B was frequent re-hospitalized with prolonged LOS during 2 nd – the 3 rd trimester due to acute lung edema. We found a mid-diastolic murmur grade II/IV at the apex. Electrocardiography (ECG) of case A: sinus rhythm, left atrial enlargement (LAE), while case B: AF rapid response. The echocardiography of case A revealed severe MS, while case B revealed severe MS, moderate tricuspid regurgitation and, a high probability for PH. Those findings support the diagnosis of severe mitral stenosis and rheumatic heart disease in pregnancy, then they were programmed to do balloon mitral valvuloplasty (BMV) in 3 rd trimester. Discussion: The BMV was performed, and succeeded in case A reducing the mitral valve pressure gradient (MV PG) from 24.7mmHg to 12.1mmHg by using local anesthesia along the procedure, while in case B specifically done BMV with general anesthesia due to supraventricular tachycardia (SVT) and pulmonal congestive during procedure, reducing the MV PG from 17.7mmHg to 8.6mmHg, as well as improvement in symptoms, up to pregnancy was terminated as obstetric indication by SC on 36-37 weeks' gestation in both cases. The baby born was healthy with weights of each case 2340gr and 2630gr. Conclusion: Mitral stenosis in the peripartum needs to be managed by interprofessional collaboration properly, to decrease the risk of morbidity and mortality for the mother and fetus.


INTRODUCTION
The diagnosis of mitral stenosis (MS) is considered clinically significant if the valve area is ≤ 1.5 cm 2 .This narrowing impedes the normal blood flow from the left atrium to the left ventricle, leading to a range of symptoms and potential complications.While MS can occur at any stage of life, peripartum MS specifically refers to the development or exacerbation of the condition during the period surrounding childbirth.The reference measurement of MS severity is planimetry.Doppler-pressure half-time is less reliable but can be used during pregnancy.Based on the Registry of Pregnancy and Cardiac Disease (ROPAC), the prevalence of MS during pregnancy showed 70% in mild MS, 39.2% in moderate MS, and 19.8% in severe MS.The mortality itself is higher in low-middle-income countries like Indonesia. 1,2 women with pre-existing MS, pregnancy-related physiological alterations can worsen the narrowing of the mitral valve and result in peripartum complications.Peripartum MS poses unique challenges to both the mother and the unborn child.The compromised blood flow through the narrowed valve can lead to symptoms such as shortness of breath, fatigue, palpitations, and edema.In severe cases, it may lead to heart failure or other serious cardiac events, potentially affecting both the mother and the baby's well-being.The complications which can be found in the fetuses; are 20-30% prematurely born, 5-20% intrauterine growth restriction (IUGR), and fetal death found 1-5%.In this context, awareness of the condition's potential risks, early detection, and timely intervention are critical in ensuring the best possible outcomes for both the mother and the baby.

CASE
We presented both pregnancy mothers were referred to the cardiovascular department.who had severe MS and developed worsening complaints in third trimester of pregnancy.All patients underwent balloon mitral valvuloplasty (BMV) before labor.These were performed in Dr. Kariadi General Hospital, Semarang, Indonesia.The procedures were performed BMV with femoral approach, and Case A under local anesthesia (lidocaine injection agent 2% 10 ml) to do procedure BMV, within specialty in Case B due to frequent SVT and pulmonal congestive during procedure, then under general anesthesia using fentanyl Inj.1-2 mcg/kgbw, then adding the muscle relaxant agent rocuronium 0.6-1.2mg/kgbw followed by rapid sequencing intubation and to maintenance hemodynamics by sevoflurane 1 MAC (2%) MAP targeted 70mmHg.The BMV was succeeded in both cases; by reducing the mitral valve pressure gradient from 24.7mmHg to 12.1mmHg (Case A) and reducing the mitral valve pressure gradient from 17.7mmHg to 8.6mmHg (Case B).

Case
The evaluation of transthoracic echocardiography post-procedural of BMV with quantification for mitral valve area by planimetry 1.9cm 2 and mean pressure gradient 10mmHg (Case A), whereas the quantification mitral valve area by planimetry 1.77cm 2 and mean pressure gradient 9mmHg (Case B).Physiological changes in pregnancy begin to occur in the first trimester, but these changes will continue into the second and third trimesters.These physiological changes include increased blood volume, cardiac output, stroke volume, heart rate, and decreased blood pressure and peripheral vascular resistance.Cardiac output increases by 40% starting in the 5th week of gestation and reaches a peak at 32 weeks.These physiological changes will reach their peak between the second and third trimesters which can cause symptoms if the pregnant woman has heart disease.These physiological changes will disappear within 3-6 months after delivery. 3,4,5ring labor, the mother's hemodynamics are affected by several factors, including pain, method of delivery, and analgesia.Cardiac output increases to 30% in the 1st stage and up to 80% immediately after labor.The increase in cardiac output caused by an increase in stroke volume will continue until 24 hours after labor.Where each uterine contraction, 300-500 mL of blood is auto-transfused from the placenta into the maternal circulation.In addition, systolic and diastolic blood pressure increases with each uterine contraction.Maternal hemodynamic changes dramatically in the first 24 hours postpartum.Preload increases after loss of inferior vena cava pressure due to uterine compression.This causes in the postpartum period, women with heart disease will experience the risk of symptoms of heart failure.In addition, pregnancy is hypercoagulable, thus making women more prone to thrombosis.The risk of thrombosis is highest within 6 weeks after delivery, and the increased risk occurs up to 12 weeks after delivery. 6,7

Mitral stenosis in Peripartum
In general, patients with moderate to severe mitral stenosis tolerate pregnancy poorly.Increased plasma volume and heart rate will worsen the patient's condition so the NYHA functional class in pregnant women will increase by at least 1 point.Patients who are initially asymptomatic may become NYHA II, and patients who are initially NYHA II may become NYHA III.Mitral stenosis in women often becomes symptomatic during pregnancy when cardiac output increases to 70%.Referring to the Gorlin formula, an increase in cardiac output of 1.7 times will increase the transvalvular gradient by 2.89 times.So if the prepregnancy gradient of 4mmHg can become 11mmHg during the second trimester of pregnancy, this will cause symptoms.The highest risk of mortality for mothers during childbirth and immediately after delivery.The sudden increase in preload after delivery, due to autotransfusion from the uterus, can overwhelm the maternal circulation and cause severe pulmonary edema.This autotransfusion lasts for approximately 24-72 hours after delivery. 8sk stratification is especially important in pregnant women with heart disease.Where the Zahara risk score is used for pregnant women with congenital heart disease, while for pregnant women with heart valve disease and artificial heart valves using the Carpreg score.World Health Organization (WHO) classifies pregnant women with congenital and acquired heart disease into 4 classes, from low to high risk.Patients with WHO class 4 are at greatest risk, in whom pregnancy is contraindicated because of the high mortality rate. 9

Intervention
Counseling for pregnant women regarding percutaneous intervention therapy needs special attention and approach.
During pregnancy, percutaneous intervention on the mitral valve is recommended after entering 20 weeks' gestation with consideration for pregnant women with NYHA class III/IV and/or estimated systolic pulmonary artery pressure (PAP) ≥ 50 mmHg, who have received optimal treatment without any contraindications.Percutaneous intervention is recommended after entering 4 months of gestation in the second trimester.By this time, organogenesis is completed, the fetal thyroid is inactive, and the volume of the uterus is still small, so that there is a greater distance between the fetus and the chest wall than in the following months.Close monitoring is required within a few days postpartum.The prognosis will depend on the risk of developing stenosis and restenosis progression after intervention, so regular follow-up is necessary. 9

Anesthetic Agents in the Peripartum
Maintaining hemodynamics after induction of anesthesia is a challenge.Anesthetic management of patients with heart failure especially in peripartum whether general or regional anesthesia is appropriate remains unclear. 9Therefore, only general anesthesia is indicated for patients with severe cardiac and respiratory failure. 10,11We considered these in Case B where the patient suffered severe mitral stenosis and had a high probability of Pulmonary Hypertension by transthoracic echocardiography. 12Any anesthetic agent can lead to a decrease in afterload, potentially resulting in hemodynamic collapse.Therefore, midazolam and fentanyl are commonly preferred in patients with cardiac dysfunction because of the relatively slow onset of the anesthetic effect and low effect on hemodynamics, respectively. 13re are some pros and cons of using general anesthesia in pregnancy.The pros of using general anesthesia in pregnancy include ensuring control and stability (the patient remains completely unaware and immobile during the procedure), effective pain management, and maintain stable maternal vital signs during the procedure.Meanwhile, the cons are potential risks to the developing fetus (especially at first trimester), could trigger preterm labor, and the risk of uterine atony.It has been illustrated in both cases a 42year-old and 33 years old woman were found with the chief complaint of shortness of breath.Shortness of breath has been getting worse since the last 3-4 months ago, previously mild shortness of breath could be reduced with rest.This indicates the progressive course of the disease.Dyspnea on exertion accompanied by decreased activity tolerance, and edema in both legs, are symptoms consistent with heart failure with mitral stenosis.Based on the data of history and physical examination, the patients were diagnosed with functional class III and IV chronic heart failure due to valvular heart disease.This valvular heart disease has probably been around for a long time, this can be seen from the enlargement of the left heart.Narrowing of mitral valve and increased HR of pregnancy limits LV filling resulting in decreased stroke volume and cardiac output.As a result, the heart cannot cope with situations warranting increased metabolic demand or increased blood volume.Patients with these conditions are at high risk during the peripartum.
The consideration to choose general anesthesia in case B is based on its complexity.Basic goals for anesthetic management of MS patients are to avoid tachycardia; revert acute AF to sinus rhythm; avoid aortocaval compression; maintain adequate venous return and systemic vascular resistance (SVR); and prevent increased pulmonary vascular resistance.The Case B, needs to be aware of atrial fibrillation which may result in hemodynamic deterioration and decreased cardiac output in these patients with poor cardiac reserve and hence requires faster intervention and cautious use of medication to avoid harm to the fetus and to prevent thromboembolism.
During BMV procedure, the patient experienced frequent SVT and pulmonal congestive.Due to its complexity, emergency, duration, and high degree of immobility of procedure BMV, general anesthesia using fentanyl was chosen in case B to avoid further complications during procedure.The advantages of using fentanyl is increasing pulmonary artery pressure, increasing the RV overload (by TAPSE 21mm), which benefits to LV unloading, and decreasing congestion with optimize diffusion.While monitoring fetal bradycardia was not found.There are no controlled studies examining the best type of anesthesia in these patients and guidelines are lacking.It's crucial to thoroughly assess the riskbenefit ratio and consider alternatives that minimize risks to both mother and fetus, education and information is really needed to patient and family.Selection of anesthesia technique, intensive hemodynamic monitoring, and slow and cautious titration of oxytocic drugs are important in decreasing the perioperative fetomaternal morbidity and mortality. 14,15fore and after BMV, a mitral valve gradient was examined by tapping the right heart, with the results of BMV reducing the mitral valve gradient and transthoracic echocardiography post-BMV was also performed and the planimetric mitral valve area increased from before BMV.Clinically the patient was improving, the pregnancy could be maintained as optimally as possible until the baby's weight was considered to be born.At 36 and 37 weeks of gestation, both patients were hospitalized for a caessarean section procedure without any complications for the mother and baby born.

CONCLUSION
Mitral stenosis in the peripartum needs to be managed by interprofessional collaboration properly, to decrease the risk of morbidity and mortality for the mother and fetus.

Figure 1 .Figure 2 .
Figure 1.Case A revealed severe mitral stenosis, while Case B revealed several mitral stenosis with moderate Tricuspid regurgitation and high probability for pulmonary hypertension (Pre-Procedural of BMV)

Table 1 .
Summary of case series can be described below this