Children with hemodynamically significant congenital heart disease can be identified through population-based registers

Figures & data

Table 1 The Medical Products Agency: Swedish recommendations for the treatment of children with hemodynamically significant congenital heart disease with palivizumab

Target group	Recommendation
1	Children below 6 months of age with a significant heart disease and Down syndrome or other chromosomal abnormality or known immunodeficiency or significant syndrome
2	Children below 6 months of age with left-to-right shunt
3	Children below 12 months of age with a univentricular heart or children where the primary pulmonary blood supply comes from a Glenn anastomosis
4	Children below 12 months of age with structural heart disease plus lung disease where surgical repair of the heart repair is performed before 1 year of age
5	Children below 12 months of age with idiopathic pulmonary hypertension or with pulmonary hypertension despite former heart surgery
6	Children below 12 months of age with a diagnosis of cardiomyopathy and heart failure who are on triple medication or have had a heart transplant before the age of 2 years

Table 2 Condensed description of algorithm*

Interpretation group	Inclusion diagnosis	Inclusion procedure	Inclusion drugs	Exclusion criteria/on	Age restriction (months)
1	Q86, Q87, Q89, Q9, D80, D81, D82
2	Q210 Q211 Q212 Q214 Q250	FHB, FHC, FBC FFC FHD, FFD, FBC FDD10, FDD13, FDD20 FDE	C03, C09	Surgery <1 month	<6
3	Q204, Q226, Q234, Q252 Q224 Q232 Q203 + Q205 + Q220 + Q210 Q220 + Q245 Q225 + (Q220, Q221)	FAE FAE FBL40, FDA, FAE00, FAE10			<12
4	Q2 + (Q30, Q31, Q32, Q33, Q34, E84, J38, J41, J42, J43, J44, J47, P24, P25, P27, P28 except P283 and P284)	FA, FB, FC, FD, FE, FF, FG, FH, FI, FJ, FK, FL, FM, FN, FO, FQ, FR, FS, FT, FU, FV, FY, FZ			<12
5	I27, P293B	FA, FB, FC, FD, FE, FF, FG, FH, FI, FJ, FK, FL, FM, FN, FO, FQ, FR, FS, FT, FU, FV, FY, FZ		Only surgery, no diagnosis No diagnosis after surgery Only one diagnosis No surgery and diagnoses only <2 months	<12
6	I42 + I50 FQA, FQB, Z941, Z942, Z943		C03C, C03D, C03CA, C09A, C09C, C07A, C01A, C01CA, C01CE, C01CX, C02KX01, G04BE03, B01AC11	<3 different groups (C03, C09, C07, C01, C02, G04, and B01) during a 3-month period	<12 <24

Notes: All criteria on the same row must be fulfilled (logical “and” between columns). If the criteria in a row within an interpretation group are fulfilled, the child is included (logical “or” between rows). Comma (,) means logical “or” and plus (+) means logical “and”.

* For definitions of the codes, see the “Supplementary material” section.

Table 3 Children with hemodynamically significant congenital heart disease identified by the proposed algorithm (yearly prevalence per 1,000 live born)

Year	2005	2006	2007	2008	2009	2010	Total
Prevalence (per 1,000 live born)	1.4	1.6	1.3	1.6	1.8	1.7	1.6

Table 4 Distribution of children in studied interpretation groups and distribution of children with chromosomal aberrations

Interpretation group	N (%)	Chromosomal aberration (%)
2	365 (39.3)	40
3	201 (21.7)	8
4	137 (14.8)	18
5	127 (13.7)	14
6	14 (1.5)	0
Combination, 2–6	84 (9.1)	31
Total	928 (100.0)	25

Figure 1 Distribution of children treated with palivizumab in relation to identification by the algorithm and whether the case met the indications given in the recommendations for treatment.