INNOVATIVE ORGANIZATIONAL STRATEGY FOR CRITICAL CONGENITAL HEART DISEASE
We described innovative organizational strategy for neonates who have prenatal diagnose of critical congenital heart disease (CCHD) with focus on earliest surgical repair, using autologous umbilical cord blood. Since September of 2009 to August of 2012, 390 neonates with CCHD were operated at our institution. For that period 50 neonates underwent the new strategy. The neonates were admitted to the cardiac department during an hour after birth. No patients required ICU admission, interventional procedures, mechanical ventilation or medications before surgery that result in significant positive economic effect compared with conventional approach. Mean age at operation was 6). Mean volume of harvested cord blood was 85±24 ml (50–140). Forty (80%) neonates underwent open cardiac surgery without homologous blood transfusion.
Key words: Prenatal diagnosis, neonatal cardiac surgery, autologous umbilical cord blood.
The critical congenital heart disease remains the leading cause of death in neonates. Thirst of all it concerns patients with severe congenital heart disease who need immediate surgery after birth . The critical congenital heart diseases include transposition of the great vessels, critical aortic coarctation or full interrupted aortic arch, critical aortic stenosis, pulmonary atresia or tetralogy of Fallot with pulmonary atresia, critical pulmonary atresia, hypoplastic left-heart syndrome, total anomalous pulmonary venous connection and others .
Nowadays a critical congenital heart disease can be effectively diagnosed in fetus at starting of midpregnancy .
Due to the peculiarities of fetal hemodynamics in particular, fetal shunts and placental type of oxygenation, the most of fetal with CCHD are relatively in compensated state. After birth and in the early pulmonary respiration the CCHD determines the hypoxemia development and heart failure which lead to neomortality. Surgical management of CCHD during newborn period is one of the most complicated cardiosurgical brunches. Post-surgical lethal outcome among patients of this group is 7–20% . CCHD prenatal diagnosis in Ukraine has become relatively recently and its fast development in lately years has influence on CCHD surgical management approach and results. If CCHD is prenatal detected the doctors have possibilities of medical care coordination to pregnant women and newborns involving certain specialists. Also it makes possible to provide medical care in a timely and professionally to achieve optimal treatment results. So since 2009 Ukrainian Children's Cardiac Centre have been starting the cardiosurgical program of the first life hour's patients with prenatal diagnosed CCHD. Taking into account the tendency to increase of the accessibility and CCHD fetal diagnosis quality, its influence the neonatal cardiological care will increase in the future. Prenatal diagnosis also give the unique chance to provide for the need of surgical treatment in the early period and to take an autologous cord blood samples which may be effective alternative to homologous blood components for transfusion during the surgery with cardiopulmonary bypass using. By this way is decided the question of safety usage of homologous blood components for newborns.
Material and methods
There was retrospective study analysis of the prenatal echocardiography database in fetus and newborns with CCHD who were operated in Ukrainian Children's Cardiac Centre from 2009 September till 2012 August.
The study was conducted upon of our medical establishment bioethics committee authorization. For each case was received parents` written consent to operative treatment. During that period were operated 390 newborns. 50 patients with prenatal detected CCHD among them were included into the cardiosurgical program of the children's first life hours. This program includes:
1. Repeated fetal echocardiography at 36 weeks' gestation to CCHD diagnosis confirmation in fetus.
2. Screening of pregnant women for arthropodborne infections and prescribing of medical consultation in maternity clinic in Kiev.
3. Children's birth planning with CCHD in the nearest Kiev's maternity clinic to Children's Cardiac Centre during a workweek in the morning time according the centre operative schedule with booking before.
4. Autologous cord blood sampling while a labour and testing of samples in blood transfusion centre according to Standards for Blood and Blood Components.
5. Immediate delivery of a newborn to the cardiac department.
6. Definitive repair of CCHD during the first life hours.
7. Using autologous cord blood in cardiopulmonary bypass surgery for transfusion.
Pregnant women consulted on the recommendation of perinatal centers or individually. The majority of the first consults were from 22 till 35 weeks' gestation.
After CCHD confirmation in fetus a pregnant woman had a conversation with specialist about CCHD specifics and possible variants of stage or just after birth repair. An important aspect touched in that conversation was explanation of need for donated blood components using and also to show the possibility of CCHD definitive repair during the first life hours using ACB.
In cases when parents accepted this treatment way the parents' written consent to operative repair, sampling and using of ACC while the surgery must be got. After that the pregnant woman with CCHD conclusion in fetus was directed into maternity clinic to determine gestational age, gestation course peculiarities and approximate labour term. Repeated fetal echocardiography had been conducted just before admission to maternity clinic as rule at 36–37 weeks' gestation. It was obligingly to pregnant woman of arthropod-borne infections examination and performed during this visit of Cardiac Centre for determination sampling possibility and autologous cord blood using (in compliance with the order of the Ministry of Health of Ukraine on 01.08.2005 №385 «About infectious blood safety and blood components».
Deliver babies were conducted in collaboration with the closest to our institution maternity clinics in Kiev. It gave the opportunities for all pregnant women apart of registration at place of residence to labor babies with CCHD close to special cardiac institution, to cut the time between birth and surgery and thus to reduce the transportation risk of newborn. The suggested organizational approach is presented in Table 1. After hospitalization into maternity clinic was performed obstetric examination to determine approximate labour term and report this term to the Cardiac Centre personnel. Generally a labor is planned in the morning time of workweek (6.00–8.00 a.m.) that makes possible to form the Centre's operative schedule ahead of time.
In exceptional cases after obstetric indications was performed cesarean operation that significantly eased cardiac operation planning. An obstetrician-gynaecologist informed Cardiac Centre's specialists about labor before 1.5–2 hours till the second labor study to birth preparing baby's with CCHD. After that the special ambulance crew of Cardiac Centre came to maternity clinic for cord blood sampling and newborn moving.
Just after baby birth in physiological way or cesarean operates and the cord cutting off by the cardiac centre's doctor who have special proficiency and took the cord blood sample in utero into closed plastics system for sampling with CPDA according to aseptic regulations.
The packages with cord blood were weighted and placed into heat-insulating box. The doctor who had taken the cord blood before recorded supporting documentation. After baby birth the neonatologist made the first examine of newborn and assessment of the state after Apgar score and in case of the need delivered appropriate care. For safety transportation newborn's with CCHD to Cardiac Centre was necessary the vital functions screening (ECG, AP, Arterial oxygen percent saturation). The portable cardiac monitor was used in that situation. In additional was set on the peripheral arterial line to emergency procedure if a physical condition decline during the transportation. To objective assessment of newborn state was evaluated acid-base balance and gas composition in peripheral blood by Blood Gas Analyser in maternity clinic. After newborn's examination by cardiologist the consultative notes were written into labor and delivery record for
moving to Cardiac Centre. The transportation baby was by an emergency ambulance under the care of resuscitator with regular control of total state and cardiomonitoring data.
Just after arriving to Cardiac Centre the echocardiography was performed to newborn for prenatal diagnosis confirmation and functioning fetal shunts` evaluation that determine a stable state prognosis in patient.
In case of need for the purpose of heart-disease peculiarities were conducted the computer tomography of the heart and vessels. All of the patients were performed the screening neurosonography to exclude of underlying condition and birth injuries which may be contraindication to surgery with cardiac bypass. After all necessary diagnostic procedures the newborns with CCHD were moved into operating room, avoiding intensive care unit where pre-surgical anaesthetic preparation of patient was started. Harvested autologous umbilical cord blood was delivered into autotransfusion department to assessing of availability to transfusion, group belonging and arthropod-borne infections antibodies express-test. The blood was also revised for sterility. After marking appropriate notes on package with blood and marking its autologous the blood was used for perioperative transfusion providing. Besides of that was conducted serological component selection of homologous blood in case of additional transfusion.
Results and discussion
390 neonates with CCHD were operated at Cardiac Centre during that period. To 50 (12.8%) among them with prenatal diagnosed was used this strategy and described in Table 2. Twenty nine (58%) patients were born in physiological way; twenty one was born in cesarean operates according obstetrical data. Usually the assessment after Apgar score between 1 and 5 minutes was stated at 7–8 points, which shows satisfactory total condition in neonates. Average volume of harvested cord blood was 85±24 ml (50–140). As a standard blood bag designed to more volume so the volume adaptation of blood fraction to prognosticative harvested ACB volume in the ratio of CPDA-1: ACB as 1:5 is important. Neonates were moved to Cardiac Centre during the first hour after birth. No patients required pre-surgical medical treatment and intensive care.
Average newborns' age by the operation start was 3.9±1.1 hours. Definitive repair was performed after the method designed by the Centre according to a cardiac defect. There was used the standard bypass protocol for neonates, mild hypothermia and hypothermic crystalloid cardioplegia. Average time of aorta clamping and bypassing were no different from a standard time of repair for the same cardiac defects in later years . In the early postoperation time the patients show tendency to cutting time of artificial lung ventilation and resuscitation.
The largest group was patients with transposition of the great arteries (TGA). This cardiac defect has high sensibility and specific of prenatal diagnostic and as a rule not associated with genetic syndrome and can be one-stage definitive repaired. Due to the fact that patients with TGA required the arterial switch operation in the first life hours and were in satisfactory condition by the time of arriving to the Centre and just before the operation we gave up balloon atrial septostomy as the first surgical treatment stage. This treatment model has shown some advantages such as avoiding risk of potential endovascular surgery complications
(X-irradiation, contrast medium infusion, thigh vein thrombosis, cardiac chambers perforation etc.) and significant economical effect because this endovascular procedure is expensive. In addition according latest
data, delayed planned surgery in newborns with CCHD has not benefit and even can raise a child mortality rate and number of complications . Such complications as respiratory failure with prolonging respiratory support, slowdown in weight increasing, sepsis, chronic lung diseases, necrotizing ulcerative enterocolitis and acute renal failure followed by delayed operation . Forty (80%) neonates were operated exceptionally using ACB and without homologous blood components.
During the operation ACB was used to fill the first volume of bypass and transfusion. Additionally donated packed red cells were used in 10 patients to iatrogenic anemia correction.
A raise availability and prenatal echocardiography quality in perinatal centers lead to increase number cases of diagnostics such cardiac pathology as may be definitive repaired during newborn period. An implementation of described organizational system lets to conduct surgical repair of prenatal diagnosed CCHD during first 3–4 newborn life hours routinely without pre-medical therapy and salvage endovascular procedures. The important is an effective and close cooperation between obstetric department and neonatal cardiosurgical clinic as early as the prenatal stage because this is the very thing that make possible to fast and high level medical care to newborns with CCHD. Autologous cord blood is effective and safety alternative to using of homologous blood components in neonatal cardiosurgery. Average volume of harvested
cord blood was 25–30% of circulation blood volume of newborn and was suffice for cardiosurgical intervention in the most cases. Designed by us algorithm potentially may be used to all of prenatal diagnosed of critical congenital malformation that is to be surgical treated in neonatal period. This branch is the innovation not only in Ukraine but also in the World.
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