Table 3.

Characteristics of Patients With PH That Was Diagnosed Before or After Diazoxide Initiation

Patient	Cardiac Anatomy	Respiratory Comorbidities	Age at Diazoxide Initiation (d)	Age at Echo With PH (d)	Diazoxide dose at PH Diagnosis, mg/kg/d	Echo Criteria for PH	Time from Diazoxide to Echo With PH, d	Age at Diazoxide Discontinuation, d	Age at Repeat Echo, d	PH Resolved
Patients with PH on echo after diazoxide initiation
1	Normal	Respiratory failure	6	23	16	RVPE > 1/2 systemic (by TRJV), flattened ventricular septum	17	23	71	Yes
2	Large PDA	Respiratory failure, prematurity (26 wk), severe IUGR	Unknown	65	15	RVPE > 1/2 systemic (by PDA), flattened ventricular septum	Unknown	62	100	Yes
3	Large PDA	Respiratory failure, prematurity (27 wk)	54	83	6.25	Flattened ventricular septum	29	83	88	Yes
4	ALCAPA		4	6	11.5	RVPE > 1/2 systemic (by TRJV), flattened ventricular septum	2	7	NA	Unknown
5	Conoventricular VSD	Respiratory failure, prematurity (30 wk)	19	336	15	flattened ventricular septum	317	Unknown	392	Unknown
6	PDA after ligation	Prematurity (30 wk)	142	145	10	RVPE > 1/2 systemic (by PDA)	3	1607	NA	Unknown
7	PDA after ligation	Prematurity (28 wk)	Unknown	246	10	flattened ventricular septum	unknown	168	NA	Unknown
Patients with PH on echo before diazoxide initiation
8	RCM	Respiratory failure, prematurity (27 wk)	44	6		Flattened ventricular septum	−38	65	128	No
9	Normal	Meconium aspiration, respiratory failure	6	1		–a	−5	239	NA	Unknown
10	CAVC, small PDA	Respiratory failure, influenza A, chylothorax	142	0		Flattened ventricular septum	−142	150	296	No
11	Large PDA	Respiratory failure	8	5		Suprasystemic RVPE (by TRJV)	−3	11	36	Yes
12	Conoventricular VSD, PDA, RLPV/LLPV stenosis, PAPVC– LUPV to innominate vein, isthmus hypoplasiab		24	8		Flattened ventricular septum	−16	37	187	Yes
13	Small PDA	Respiratory failure	1	1		–a	0	31	31	No
14	Pulmonary valve stenosis	OSAc	12	4		Flattened ventricular septum	−8	794	NA	Unknown

Abbreviations: ALCAPA, anomalous left coronary artery from the pulmonary artery; CAVC, complete atrioventricular canal; echo, echocardiography; IUGR, intrauterine growth restriction; LLPV, left lower pulmonary vein; LUPV, left upper pulmonary vein; NA, not available; OSA, obstructive sleep apnea; PAPVC, partial anomalous pulmonary venous connection; PDA, patent ductus arteriosus; RCM, restrictive cardiomyopathy; RLPV, right lower pulmonary vein; RVPE, right ventricular pressure estimate; TRJV, tricuspid regurgitant jet velocity; VSD, ventricular septal defect.

^aEchocardiography performed at outside institution and images unavailable for review.

^bTrisomy 21.

^cBeckwith-Wiedemann syndrome.