Patient A, a 20-year-old, first time pregnant woman with a history of congenital heart disease (CHD), corrected transposition of the great vessels via VSD repair, ASD (in childhood), tricuspid valve replacement (6 years ago), MR, grade I PR, grade II mitral and tricuspid valve regurgitation, and grade II heart failure based on the NYHA classification, was registered under the surveillance of her primary care physician at 12 weeks pregnancy. The pregnancy was planned and wanted.
Over the course of the pregnancy the patient was monitored by her family doctor and consulted by obstetric, cardiology, and heart surgery specialists. Taking into consideration preexisting heart problems and previous surgical interventions, the patient was recommended a treatment with anticoagulants, VKAs (Acenocoumarol) during pregnancy. The heart surgeon recommended maintaining international normalized ratio (INR) values between 2.5 and 3. Thus, throughout the pregnancy the prescribed dose of anticoagulants varied between 6 and 7mg of Acenocoumarol. In addition, at every antenatal visit coagulation testing was carried out for monitoring the effectiveness of the anticoagulant treatment necessary for patients with anamnesis of heart valve replacement. During the pregnancy, the patient had two echocardiograms, the results of which demonstrated satisfactory functioning of the heart.
Patient A was supervised during the antenatal period by her family doctor, showing up for all planned medical visits. Five ultrasound exams were conducted over the course of the pregnancy, at 13, 15, 21, 28, and 34 weeks. In all cases, development and the intrauterine condition of the fetus was assessed as satisfactory. At 28 weeks pregnancy, the patient was urgently hospitalized in a tertiary healthcare institution with the following diagnosis:primigravida, primipara, imminent premature birth, CHD, corrected transposition of the great vessels via VSD repair, ASD, tricuspid valve replacement, MR, grade I PR, grade II mitral and tricuspid valve regurgitation, and grade II heart failure based on the NYHA classification. Monitoring and consultation from a cardiologist took place and the dose of administered cardiometabolic medication (2mg Acenocoumarol- 3 times) was corrected.
Even though delivery was recommended to take place in atertiary level maternity hospital following leaking of amniotic fluid and the onset of regular uterine contractions, the patient urgently went to a primary level maternity unit in her place of residence. The following diagnostics were recorded: 39-weeks pregnant, primipara, premature rupture of membranes (PROM), longitudinal position of the fetus, cranial presentation, anterior variety, position II.
The first period (4 hours 30minutes) and the second (30minutes) of birth took place physiologically, without signs of fetal suffering, and without the utilization of fetal vacuum extraction and forceps. At birth, the newborn was in a satisfactory conditionwith 8/9 on the Apgar score, had clean, pale skin and mucous membranes. The neurological status of the newborn was assessed as satisfactory. There were no pathological ocular symptoms. The head was in a dolichocephalic form, and a 3x3cm cephalohematoma wasobserved in the occipital region.
The postpartum period was complicated on the second day following birth with the appearance of a hematoma in the region of the external genital organs, as well as hemodynamic disorders. The patient was transferred to a tertiary level healthcare facility, where the following diagnostics were established: day four postpartum, condition following natural childbirth, hematoma in the region of the internal and external genital organs, grade II iron deficiency anemia, CHD, corrected transposition of the great vessels via VSD repair, ASD, tricuspid valve replacement, MR, grade I PR, grade II mitral and tricuspid valve regurgitation, and grade II heart failure based on the NYHA classification. Laboratory readings indicated low prothrombin − 36%, but the level of fibrinogen could not be detected. During her stay in the intensive care unit, she was prescribed an anticoagulant treatment and a blood transfusion. On the sixth day following her admittance, she was discharged in a satisfactory condition.
Twenty-two hours after birth, the newborn was in an aggravated condition;it suddenly became restless, refused to suck, and had a painful scream and moan. The newborn’s skin turned paler, muscle hypertonia was observed, breathing was difficult, and it had a SaO2 of88-90%.
Twenty-four hours after birth, the newborn’s condition became extremely critical. It was very pale. Regarding its neurologic condition, it had a week reaction to physical examination. The newborn went into a coma and began to convulse. Its head grew in volume with a cranial circumference of 39.4cm (4.4cm greater than at birth) and at the level of the occipital bones a fluctuatingcollection of fluidwas observed.SpO2 was not determined, frequency of heart contractions was 110 per minute, respiratory rate was 38–40 per minute, and capillary recovery time was > 3 seconds. A complete blood count (CBC) showed the following: hemoglobin − 87g/L, erythrocytes − 2.9x1012cells/L, hematocrit − 32%, leukocytes (WBC) − 28.8x103/microL, metamyelocytes – 2%, unsegmented – 10%, segmented – 50%, eosinophils – 0%, lymphocytes – 36%, monocytes – 2%. The newborn was given vitamin K – 2mg, volume expansion with 0.9% NaCl − 10ml/kg, dopamine − 10mcg/kg/min increased to 15mcg/kg/min, packed red blood cells (PRBCs) (A (II) Rhnegative)-10ml/kg,and fresh frozen plasm (FFP) − 10ml/kg. The child’s condition stabilized, SaO2 increased to 97–98% and frequency of heart contractions grew to 115–120 per minute. The skin grew pinker.
The newborn was transferred to an intensive care unit in a tertiary healthcare facility, where its condition was assessed as profoundly serious. It was moaning.SaO2 without the administration of O2 was73-74%, thus requiring artificial ventilation of the lungs with fraction of inspired O2 at 30%. SpO2 increased to91-93%. The skin was pale, capillary recovery time was > 3 second, and arterial blood pressure was 25/16/20. A fluctuating collection of fluid localized in the parieto-occipital sulcus spreading to the cervical region was observed. The newborn exhibited slow photoreaction of pupils, hypotonia, hyporeflexia, and hypodynamic heart function. Hemoglobin was 110g/L, erythrocytes − 3.4x1012cells/L, hematocrit − 33%, WBC- 45 x8x103/microL, absolute neutrophil count(ANC) 35100, immature-to-total ratio − 0.25, platelets − 170 x 103/microL, acid-base balance - pH 7.04, pCO2 16.8, pO2–54.8, plasma bicarbonate − 7.6, base deficit − 24.4. Prothrombin time (PT) was 38 seconds, activated partial thromboplastin time (APTT) − 1 minute 54 seconds, fibrinogen − 1.8gm/l, prothrombin − 46%. The newborn received volume expansion therapy with 0.9% NaCl − 10ml/kg, cardiotonic dopamine therapy- 15mcg/kg/min, homeostatic therapy with vitamin K − 2mg intravenously (IV), correction of posthemorrhagic anemia withPRBCs- 15ml/kg. Following the transfusion, hemoglobin increased to 145, erythrocytes − 4.5, and hematocrit − 0.44, respectively.
Forty-three hours after birth, clonicseizureswere registered, bloody content was eliminated from the gastronomy tube. Macrohematuria, melena, hematemesis, and anuria were observed. Cranial circumference was registered at 40cm (at birth it was 35cm). The newborn suffered a profound cerebral coma. It was in an agonal state. The CBC showed hemoglobin levels of 105g/L and platelets − 128 x 103/microL. PT was 49 seconds, APTT − 1 minute 54 seconds, acid-base balance – pH 7.11, pCO2–28.2, pO2–39.2, plasma bicarbonate − 10. The newborn was given PRBCs(A (II) Rh negative)- 15ml/kg, FFP A(II) − 20ml/kg and vitamin k IV − 2mg. Forty-eight hours after birth the newborn died.
The cause of death based on the autopsy was determined to be neonatal hemorrhagic syndrome. Acute bleeding disorders, rectal bleeding and per diapedesis in the epicranial aponeurosis, at the level of the leptomeninges, brain, thymus, lungs, diaphragm, stomach, intestines, and kidneys were observed. Collection of blood occurred at the level of the leptomeninges and diaphragm. The amount of blood accumulated in the right lung was 10ml and in the stomach- 100ml.