Download First Balloon Atrial Septostomy in Our Newborn Unit: Learnings

First Balloon Atrial Septostomy in Our Newborn Unit: Learnings From a Case.
Yenidoğan Ünitemizde İlk Balon Atriyal Septostomi: Vakadan Öğrendiklerimiz
Hatice Güneş1 *Hakan Güneş2, İrfan Oğuz Şahin3 Özlem Bozoklu Akkar3, Çağlar Yıldız3,
Pelin Kekeç Bostancı4, Hatice Güneş4, Fatih Bolat5
1
Department of Pediatrics, Cumhuriyet University Faculty of Medicine.
2
Department of Cardiology , Kahramanmaraş Sütçü Imam University Faculty of Medicine.
3
Division of Neonatology, Department of Pediatrics, Cumhuriyet University Faculty of
Medicine.
Corresponding author: Dr. Hakan Güneş , Kardiyoloji Bilim Dalı, Kahramanmaraş Sütçü
İmam Üniversitesi Tıp Fakültesi, Kahramanmaraş
E-mail: [email protected]
Conflict of interest: There is not a conflict of interest.
Summary
Transposition of the great arteries is difficult to be diagnosed in utero and require emergent
surgical intervention. Prostoglandin E1 infusion or balloon atrial septostomy (BAS) could be
necessary in awaiting the patient to surgery. This is the first case BAS performed in our clinic.
Centers have to improve antenatal detection rates of cyanotic congenital heart defects to
decrease perioperative morbidities. Pediatric cardiologs must manage these patients in
cooperation with perinatology and pediatric heart surgery specialists and also with patients
parents.
Keywords: Transposition of the great arteries, Balloon atrial septostomy, Antenatal detection.
Özet
Büyük arterlerin transpozisyonunun in utero tanınması zordur ve acil cerrahi girişim
gerektirir. Prostoglandin E1 infüzyonu ya da balon atriyal septostomi (BAS) hasta cerrahi
işlem için beklerken gerekli olabilir. Bu vaka kliniğimizin ilk BAS yapılan vakasıdır.
Merkezler siyanotik kalp hastalıklarında antenatal tanı koyma oranlarını artırarak perioperatif
morbidite oranlarını azaltmalıdır. Pediyatrik kardiyologlar bu hastaların denetimini
perinatoloji, pediyatrik kalp cerrahisi uzmanları yanında ailelerle de kooperasyonunu
sağlayarak yapmalıdırlar.
Anahtar kelimeler: Büyük arterlerin transpozisyonu, Balon atriyal septostomi, Antenatal
tanı.
INTRODUCTION
Transposition of the great arteries (TGA) is the most common cyanotic heart defect in
the newborn period that aorta arises from the right ventricle and pulmonary artery from the
left ventricle1. Most patients present after delivery with cyanosis because antenatal ultrasound
detection is difficult2.
Patients must be evaluated immediately for a communication between systemic and
pulmonary circulation to be consistent with life while the infants awaited arterial switch
operation (ASO)3. Balloon atrial septostomy (BAS) is an emergency intervention aiming to
enlarge the atrial septal defect (ASD)4. Some recent studies has reported an increase risk of
stroke after BAS5.
Prostaglandin E-1 (PGE1) infusion is another choice to promote intra-cardiac mixing
via patent ductus arteriosus (PDA). There is a concern about adverse effects of PGE1 infusion
like apnoea, hypotension, vasodilatation, arrhythmias and pyrexia. These effects have
deleterious outcomes in intraoperative and postoperative period1,6.
Therefore, there is a controversy on the best preoperative management strategy7-9.
CASE REPORT
A term (39 weeks) male infant was born with normal Apgar score and 3400 gr
birth weight via caesarean section to a first gravida (G1P1L1) of 26-year-old woman.
Cyanosis was noticed by the pediatrician at 3rd hour of delivery at rooming-in and arterial
saturation of oxygen (sPO2) was found as %80. Infant was accepted to Cumhuriyet University
Neonatology Intensive Care Unit (NICU) due to central cyanosis and immediately evaluated
for possible reasons. In physical examination fever, tachycardia, tachypnea, respiratory and
congestive heart failure findings were not found. Hyperoxia test was negative.
Echocardiographic investigation showed TGA with ventricular septal defect, restricted
(2,4mm) atrial septal defect and small PDA (Figure 1).
Follow up in NICU without enteral feeding and without O2 support was planned.
Parents of the patient were informed about the disease. Case was consulted with the centers
could perform ASO. One center accepted the patient but for the following day due to transport
procedure. Preoperative management was planned with cooperation of the surgeon and PGE1
infusion was started at the dose of 0,05 mcg/kg/min. Emergency command center was
informed for transport. At the 2nd hour of PGE1 infusion, patient revealed bradycardia (5055/min and severe hypoxia (sPO2 %55-60). Patient was resusciated appropriately and PGE1
infusion was stopped. After vital signs recovered, emergent BAS was performed under
midazolam sedation without any complications 9 hour after birth (Figure 2).
At the end of procedure pressures of atriums was equal, ASD was enlarged to 6.7 mm
and sPO2 was increased to %88-90 from %70-75 (Figure3).
Patient was operated (ASO) at the 5th day of his life.
DISCUSSION
In the absence of apparent lung disease, cyanotic infants should be immediately
transferred to a NICU with suspicion of having a cardiac disease for evaluating possible
reasons and defining the cardiac anatomy5.
Most of the patients with TGA present after delivery with cyanosis because a minority
of patients are diagnosed in utero10. Prenatal diagnosis of TGA reduces pre-operative
morbidity and provides planning delivery in an appropriate pediatric heart surgery center6.
Antenatal diagnosis is difficult because four chamber cardiac view is generally normal and
crossing of the outflow tracts may not be appreciated with ultrasonography11. Detection rates
vary from 15% to 53%12.
Informing parents must not be disregarded during the emergent interventions. Beside
characteristics of disease and planned procedures, the difficulty and rarity of in utero
diagnosis must be expressed to parents, whom especially followed during pregnancy. On the
other side, reduced pre-operative morbidity with prenatal detection suggests the need for
strategies to increase detection rates. We think asistance of pediatric cardiogy and
perinatology specialists to each other during ultrasonographic investigation of fetal heart
could increase the detection rates of congenital heart diseases (CHDs).
In the past, balloon atrial septostomy (BAS) was routinely performed to improve intracardiac mixing while the infants awaited surgery. Recent literature has reported an increase
risk of stroke in neonates who undergo BAS, although more recent studies refute this5. BAS is
recommended in case of restrictive atrial septum and severe hypoxia. PGE1 infusion is
alternative to BAS and promote intra-cardiac mixing via PDA. Theoretically, PDA will
maintain elevated pulmonary artery pressures and prepare the left ventricle for the higher
systemic vascular resistance following the ASO1. But potentiality of serious adverse events of
PGE1 result concerns in practical use. There is not a complete consensus on preoperative
management. We think it is better to plan preoperative management strategy in cooperation
with the heart surgeon who would face intraoperative and postoperative complications.
In conclusion, cyanosis must be evaluated carefully and immediately in newborns.
Antenatal detection of CHDs like TGA is difficult but is important in reducing preoperative
morbidity. Cooperation of pediatric cardiology and perinatology specialists in screening fetal
heart could improve detection rates of CHDs. Preoperative management of CHDs that require
surgical procedures must be planned in cooperation of pediatric cardiology and pediatric heart
surgery specialists.
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Figure Legends
Figure 1: Echocardiographic view of TGA
Figure 2: Balloon atrial septostomy of patient
Figure 3: Echocardiographic view of ASD after BAS