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- 1. Diagnosis and Management of Ventricular Septal Defect (VSD) with Eisenmenger syndrome MONICA OKTARIYANTHY 1 Division of Clinical Cardiology Department Cardiology and Vascular Medicine RS DR M Djamil PADANG 2023
- 2. VSD is the most common congenital heart defect in children Asymptomatic VSD without functional complications (NYHA Class I and II) usually does not have an impact on pregnancy. 8-10% of VSD develop into Pulmonary Hypertension (esenmenger syndrom) and maternal mortality about 55% 2 INTRODUCTION Mann DL,. Braundwald’s Heart Disease. 10th ed. 2015.
- 3. Four Types of VSD Infundibular, outlet •Rare 6-8% of all VSD •at below the semilunar valve in the right ventricular outlet Membran •Most commmon 80% of all VSD •At septum membran inferior to supraventricular crista. Inlet •8% of all CSD •>> located at below the inlet valves in the inlet portion of right ventricular septum •at muscular septum, bounded by the musculature ussually in the apical, central, and outer parts of intraventricular septum Muscular, trabecular 3 th
- 4. CASE ILLUSTRATION 23♀, pregnant • 33-34 weeks shortness of breath 3 day before being admitted, •Not shrinking •Not influence by food and weather •Increase even though resting •Woken up at night due to tightness •fatique The nails turned blue • 3 month before coming to the hospital HISTORY TAKING 4
- 5. Vital sign • GA: moderate illnes • BP : 110/70 mmHg • HR 96 bpm • RR 26x/i • T: 36,8⁰C • JVP : 5+3 cmH2O COR • Ictus cordis palpable 1 finger lateral LMCS ICS VI • S1S2 reg with hardened P2 sound, pansystolic mumur grade 3/6 at the LLSB Pulmo • Simetric • Fremitus sinistra, dextra normal • Sonor • vesicular, rh -/-, wh -/- 5
- 6. Abdomen • fundus height of the uterus :25 cm • fetal HR 140- 150x/min • HIS (-). Extremities • Cyanotic (+) • edema (+) • clubbing fingers (+) 6
- 7. 7 • Hb : 16,9 gr/dL • Leucocyt : 10.420 /mm3 • Hematocryt : 49 % • Trombocyt : 178.000 /mm3 • RBG : 88 mg/dl • Natrium : 140 Mmol/L • Kalium : 3,6 Mmol/L • Kalsium : 8,2 mg/dL • Klorida : 108 Mmol/L LABORATORY FINDINGS
- 8. 8 ECG ST ,QRS rate 110x/min, RAD Extreme, P wave normal and PR interval 0.16 ", QRS 0.08", ST changes (-/-), LVH (-), RVH (+), QTc 411 msec,
- 9. 9 Echocardiography Muscular VSD measuring 18-20 mm R → L shunt with a trans-VSD gradient of 10 mmHg with Eisenmenger syndrome. TR Moderate, high probability PH. LV global systolic with good function, EF 60% (Simpson), global normokinetic, good RV contractility, Left Arch, Coa (-). Muscular VSD + PH impression.
- 11. Anamnesa Physical examination ECG, Chest Xray, echo, angiography 11 • Muscular VSD PH with Eisenmenger syndrome • TR Moderate high probability ph • G1POA0H0 Preterm 33-34 weeks
- 12. 12 MANAGEMENT • O2 5 lpm • Furosemide 2x20 mg IV • Sildenafil 3x10 mg mg po • Berraprost 3x10 mcg po • Dexamethason 20 mg IV • Termination Pregnancy • Right Heart Catheterization Therapy Plan
- 13. CHAPTER II : CASE ILLUSTRATION FOLLOW UP 13 1 st day S/shortness of breath (+) O/BP: 116/65mmHg HR 88x/mnt RR 28 X/min T : 36,5 SaO2 87% Lab: Hb 16.2, Leukocytes 13510, Ht 47, Platelets 210000,PT 10.2 (C:10.3) APTT 32.1 (C: 32.8) post transfusion A/ Muscular VSD PH with Eisenmenger syndrome TR Moderate high probability ph Post SCTPP OD -1 P/O2 5 lpm, furosemide 2x20 mg sildenafil 3x10 mg Beraprost 3x 10 mg Ceftraikson 2x 1 gr Traneksamat 3x1 IV Metoclorpramid 3x1 iv Ketoroloc 2x 1 IV 2th day S/shortness of breath ↓ O/BP: 118/78mmHg HR 61x/mnt RR 24 X/min T : 36,5 SaO2 88% Lab: AGD : PH : 7,446 PCO2 29.6 PO2 45.8 HCO3 32.1 TCO2 32.2 BE - 0.3 and Sa02 83.3 A/ Muscular VSD PH with Eisenmenger Syndrome TR moderate high probability PH Post SCTTP 0D-2 P/O2 5 lpm, furosemide 2x20 mg sildenafil 3x10 mg Beraparost 3x 10 mg Ceftriakson 2x 1 gr Traneksama inj 3x 500 mg 3th day S/shortness of breath ↓ O/BP: 110/70mmHg HR 80x/mnt RR 24 X/min T : 36,5 SaO2 88% A/Muscular VSD PH with Eisenmenger syndrome TR moderate high probability PH Post SCTTP 0D-3 P/O2 3 lpm, sildenafil 3x12.5 mg beraprost 3x40 mcg Ceftriakson 2 x 1 gr PCT 3X 500 mg
- 14. CHAPTER II : CASE ILLUSTRATION FOLLOW UP 14 4st day S/shortness of breath (+) O/BP: 130/68mmHg HR 88x/mnt RR 26 X/min T : 36,5 SaO2 75% Lab: Hb 13.8, Leukocytes 12.390, Ht 47, Platelets 167.000 Ht : 40 A/ Muscular VSD PH with Eisenmenger syndrome TR Moderate high probability ph Post SCTPP OD -4 P/O2 5 lpm, sildenafil 3x10 mg Beraprost 3x 10 mg Cefixime 2x 200 mg PCT 3x 500 mg Vit C 3x1 Tranfusion PRC 2 6th day S/shortness of breath - O/BP: 118/78mmHg HR 61x/mnt RR 24 X/min T : 36,5 SaO2 88% Lab: Hb : 15.8 Lekocytes 11.890 Platelets 1780000 Ht : 41 A/ Muscular VSD PH with Eisenmenger Syndrome TR moderate high probability PH Post SCTTP 0D-5 P/O2 5 lpm, sildenafil 3x20 mg Beraparost 3x20 mg Cefixime 2x 200 mg Vit C 3x1 3th day S/shortness of breath - O/BP: 110/70mmHg HR 80x/mnt RR 24 X/min T : 36,5 SaO2 88% A/Muscular VSD PH with Eisenmenger syndrome TR moderate high probability PH Post SCTTP 0D-6 P/Discharge sildenafil 3x20 mg beraprost 3x20 mcg Cefixime 2x 200 mg Vit C 3x1
- 15. 15 CHEST X-RAY CTR 60%, Sg AoN, SgPo prominent, CW(-), apex downward, cranialised (-), infiltrate (-), plethora (-), pruning (+).
- 16. Discussion 16
- 17. Physiological Adaptation to Pregnancy • ↑ blood volume ≈50% • ↑ CO 30-50%, max between 5th and 8th months • ↓ systolic and diastolic blood pressure • ↓ systemic arterial resistance
- 18. Hemodynamic changes during delivery • Labour • ↑ O2 consumption • ↑ baseline CO • ↑ CO and blood pressure during uterine contraction (depend on mode of delivery: epidural analgesia/SC) • Post partum • ↑ Blood shift from placenta • ↑ preload and CO significant haemodynamic changes Uterine contractions,positioning (left lateral vs. supine), pain, anxiety, exertion, haemorrhage, and uterine involution, anaesthesia and infection
- 19. CONGENITAL HEART DEFECT TYPE ACYANOTIC 19 Park, Myung K. Pediatric Cardiology for Practitioners. 5th ed; 2008
- 20. Diagnosis of VSD with Eisenmenger Syndrom Anamnesa Clinical Findings A widely split and fixed S2 and a grade 2 to 3 of 6 systolic ejection murmur at LLSB ECG RAD,RVH, or RBBB Chest X ray Cardiomegaly with enlargement of the RA RV prominent PA segment Increased pulmonary vascular marking 20 Ventricular Septal Defect Pulmonary Hypertension (Left to the Right Shunt) ECHOCARDIOGRAHY Park, Myung K. Pediatric Cardiology for Practitioners. 5th ed; 2008:
- 21. VSD to Eisenmenger Syndrome 21 Baumgartner H, Eur Heart J. 2010.
- 22. MODIFIED WHO CLASSIFICATION OF MATERNAL CARDIOVASCULAR RISK 22
- 23. Algorithm of Management VSD 23
- 24. 24 • Where these patients are contraindicated for pregnancy. • If the pregnancy continuesit is strongly recommended for patients to control regularly to the cardiologists and obstetricians during pregnancy, during delivery and the puerperium.
- 25. The class of drugs that are safe for consumption is determined for pregnant patient VSD with – Eisenmenger syndrome 25 Phosphodiesterase Inhibitor • Inhibit specificcGMP phosphodiesterase (phosphodiesterase type 5 inhibitor ) icrease the puklmonary vascular respon to inhalational and endogenous NO in pulmonary hypertension Antiplatelet therapy • Patinent with Eisenmenger syndrome are at increased risk of thrombocytopenia, deficiencies in vitamin K-depende clotting factor, but the evidence base for using antiplatelet or LMWH is less develop
- 26. 26 Cesarean delivery Cesarean delivery needs a plan In high risk lesions specialist multidisciplinary team. Preparation of Operations the chosen type of Analgesia is the Lumbar Epidural. Continuous lumbar epidural analgesia with local or opiate anesthesia, or continuous spinal opioid anesthesia can be given safely Management Delivery of VSD-PH in Pregnancy Patient Regitz-Zagrosek V,ESC Guidelines on the Management of Cardiovascular Diseases during Pregnancy, 2011
- 27. Summary Pregnancy is contraindicated in cases eisenmenger syndrome Special attention and comprehensive management are required for pregnant patients with VSD-Eisennenger (Section Cesaeran, Epidural lumbal analgesia, sterilization) One way is termination of pregnancy 27
- 28. THANK YOU 28
Editor's Notes
- A 16-year-old woman admitted to Cardiovascular Department of RSUP Dr. M. Djamil Padang with chief complaint of fever since 2 weeks before admission.
- On the second day of treatment, there were still complaints of shortness of breath, fever, and phlegm cough with blood pressure was 100/47 mmHg, with heart rate 91 bpm (beat per minute), respiratory rate 24 times per minute, temperature 37.8°C and diuresis 0.8 cc/kg/hour. On physical examination, pansystolic murmur at the apex of the heart and soft wet rales were found, while wheezing and lower extremities edema were not found. Patient was diagnosed with Possible IE. Furosemide bolus iv therapy in the patient was replaced by Furosemide drip iv 3 mg/hour, while antibiotic therapy was continued. PRC transfusions and routine blood tests, MCV, MCH, MCHC, reticulocytes, and post correction electrolyte examinations were also performed in the patient. The patient then was consulted to the Internal Medicine Department. The Internist diagnosed the patient with Normochrome Normocytic Mild Anemia ec Hemolytic ec Autoimmune Suspension/Non-autoimmune Suspension. The patient was recommended to have routine blood tests (Hb, Ht, Leukocytes, Platelets) after transfusion. The patient was consulted to the Pulmonology Department as well. From history taking, physical and other examinations, the patient was diagnosed with Community Acquired Pneumonia (CAP) and minimal dextral pleural effusion. The patient was recommended for culture and sensitivity test of sputum as well as Molecular Rapid Test (TCM) and Thoracic Ultrasound, if it is possible. The Pulmonologist gave recommendation therapy, i.e. continuing antibiotic therapy as the Cardiologist advised and nebulization of N-Acetylsistein 2x 300 mg. On the third day of treatment, there were still complaints of phlegm cough, while shortness of breath had decreased and fever had been treated, with with blood pressure was 100/57 mmHg, with heart rate 79 bpm (beat per minute), respiratory rate 22 times per minute, temperature 37.1°C and diuresis 1.2 cc/kg/hour. On physical examination, pansystolic murmur at the apex of the heart and soft wet rales were found, lower extremities edema was not found. Laboratory tests results showed normochromic anisocytosis, leukocytosis with shift to the right neutrophilia, and thrombocytopenia. Electrolyte blood tests results were calcium 8.2 mg/dL, sodium 133 mmol/L, potassium 3.4 mg/dL and serum chloride 103 mmol/L which showed improvement of hypocalcemia and hypokalemia. Treatments were IVFD RL 500 cc/24 hours, Furosemide drip iv which was maintained at 3 mg/hour, Ramipril which the dose was increased become 1x5 mg, and antibiotics therapy i.e. Ampicillin iv 4x3 g and Gentamicin iv 1x160 mg were continued for 15 days, then potassium was corrected with KCl 30 mEq. The ACE-I was planned to be up titrated, while other therapies were continued.
- On the second day of treatment, there were still complaints of shortness of breath, fever, and phlegm cough with blood pressure was 100/47 mmHg, with heart rate 91 bpm (beat per minute), respiratory rate 24 times per minute, temperature 37.8°C and diuresis 0.8 cc/kg/hour. On physical examination, pansystolic murmur at the apex of the heart and soft wet rales were found, while wheezing and lower extremities edema were not found. Patient was diagnosed with Possible IE. Furosemide bolus iv therapy in the patient was replaced by Furosemide drip iv 3 mg/hour, while antibiotic therapy was continued. PRC transfusions and routine blood tests, MCV, MCH, MCHC, reticulocytes, and post correction electrolyte examinations were also performed in the patient. The patient then was consulted to the Internal Medicine Department. The Internist diagnosed the patient with Normochrome Normocytic Mild Anemia ec Hemolytic ec Autoimmune Suspension/Non-autoimmune Suspension. The patient was recommended to have routine blood tests (Hb, Ht, Leukocytes, Platelets) after transfusion. The patient was consulted to the Pulmonology Department as well. From history taking, physical and other examinations, the patient was diagnosed with Community Acquired Pneumonia (CAP) and minimal dextral pleural effusion. The patient was recommended for culture and sensitivity test of sputum as well as Molecular Rapid Test (TCM) and Thoracic Ultrasound, if it is possible. The Pulmonologist gave recommendation therapy, i.e. continuing antibiotic therapy as the Cardiologist advised and nebulization of N-Acetylsistein 2x 300 mg. On the third day of treatment, there were still complaints of phlegm cough, while shortness of breath had decreased and fever had been treated, with with blood pressure was 100/57 mmHg, with heart rate 79 bpm (beat per minute), respiratory rate 22 times per minute, temperature 37.1°C and diuresis 1.2 cc/kg/hour. On physical examination, pansystolic murmur at the apex of the heart and soft wet rales were found, lower extremities edema was not found. Laboratory tests results showed normochromic anisocytosis, leukocytosis with shift to the right neutrophilia, and thrombocytopenia. Electrolyte blood tests results were calcium 8.2 mg/dL, sodium 133 mmol/L, potassium 3.4 mg/dL and serum chloride 103 mmol/L which showed improvement of hypocalcemia and hypokalemia. Treatments were IVFD RL 500 cc/24 hours, Furosemide drip iv which was maintained at 3 mg/hour, Ramipril which the dose was increased become 1x5 mg, and antibiotics therapy i.e. Ampicillin iv 4x3 g and Gentamicin iv 1x160 mg were continued for 15 days, then potassium was corrected with KCl 30 mEq. The ACE-I was planned to be up titrated, while other therapies were continued.