Patau and Edwards Syndromes in a University Hospital: beyond palliative care

To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes.

Methods:

Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied.

Results:

33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30–36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2–304) and 25 days (13–58), respectively.

Conclusions:

Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

Keywords:
Trisomy 13 syndrome; Trisomy 18 syndrome; Heart defects, congenital; Palliative care

	n	%
Gestational age at birth (weeks)
<30	0	0
30–36	8	24.2
>36	25	75.8
Birth weight (g)
<1000	0	0
1000–1499	6	18.2
1500–2500	23	69.7
>2500	4	12.1
Apgar score at the 5^th minute <7
Yes	5	15.1
No	28	84.9
Age on admission
At birth	26	78.8
Until 28 days	5	15.1
>28 days	2	6.1
Mother’s age (years)
<18	1	3
18–34	19	57.6
>34	13	39.4
Presence of maternal comorbidity	12	36.4
Prenatal care performed	33	100
Prenatal diagnosis
Syndrome	10	30.3
Heart disease	23	69.7

	Yes		No
	n	%	n	%
Comorbidities	22	81.5	5	18.5
Other malformations	22	81.5	5	18.5
Heart disease surgeries	4	15	23	85
Other surgeries	16	59.2	11	41.8
Invasive interventions	27	100	0	0

	n	%	Days of hospitalization (median and limits)
Death	16	59.3	14 (2–113)
Heart cause	9	56
Non-heart causes	7	44
Transfer to origin	1	3.7	22
Discharge	10	37	68 (29–304)
Oxygen dependence	7	70
Drugs for heart failure	8	80
Tracheostomy	2	20
Gastrostomy	10	100
Anticonvulsant drugs	2	20

Case	Surgery performed	Age (days)	Outcome	Days in hospital
1	PDA closure and pulmonary artery banding	83	Discharged	101
2	Pulmonary artery banding	47	Death due to sepsis	80
3	Correction of interatrial communication, interventricular communication, and PDA	41	Discharged	150
4^* * The family came by themselves from Bahia to São Paulo, with a 6-month-old baby, with no diagnosis of trisomy 18. After the diagnosis, the patient was followed-up in the outpatient clinic. PDA: Patent Ductus Arteriosus.	Correction of Atrial Septal Defect and Ventricular Septal Defect	395	Discharged	120

Sociedade de Pediatria de São Paulo R. Maria Figueiredo, 595 - 10o andar, 04002-003 São Paulo - SP - Brasil, Tel./Fax: (11 55) 3284-0308; 3289-9809; 3284-0051 - São Paulo - SP - Brazil
E-mail: rpp@spsp.org.br

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[1] *Corresponding author. E-mail: ligia42@gmail.com (L. Marçola)