Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature

Table 1 Pertinent laboratory investigations

Investigation	Patient result	Reference range
Sodium (mmol/L)	138	135-145
Potassium (mmol/L)	4.1	3.5-5.0
Chloride (mmol/L)	104	98-111
Carbon dioxide (mmol/L)	22	21-35
Blood urea nitrogen (mg/dL)	19	10-25
Creatinine (mg/dL)	0.80	< 1.28
Venous lactate (mmol/L)	1.2	< 2.1
High-sensitivity troponin (ng/L)	16	< 18
Brain natriuretic peptide (pg/mL)	472	< 50

Table 2 Cardiopulmonary exercise testing trend over time

Date of CPX	Peak HR (bpm)	Duration (min)	Peak VO2 (ml/kig/min)	VO2 at anerobic threshold (ml/kig/min)	Peak VO2 (% of age predicted)	Peak RER	Actual METS achieved	VE-VO2 slope	Peak double product	O2 pulse rest	O2 pulse peak
June 16, 2016	85 (46% predicted max)	12.0	18.2	13.5	47	1.28	5.2	28.0	11900	4	16
February 16, 2017	145 (78% predicted max)	13.5	22.4	16.4	59	1.23	6.4	25.6	22040	4	12
September 6, 2018	173 (94% predicted max)	10.5	20.1	-	58	1.20	5.7	28.8	25258	3	11
June 13, 2022	173 (96% predicted max)	9.5	19.5	-	56	1.17	5.6	22.8	21106	3	11

CPX: Cardiopulmonary exercise testing; HR: Heart rate; METS: Metabolic equivalent of task; RER: Respiratory exchange ratio; VE: Ventilatory efficiency.

Table 3 Echocardiography trend over time

Date of study	Type of study	sRV parameters	sAV valve parameters	vLV parameters	vAV valve parameters	LA parameters	RA parameters	PA pressure (mmHg)	Other details
April 13, 2016	Transthoracic	Severely reduced global RV SF. Moderately enlarged RV	Severe Reg (sAV Reg Vmax 4.17 m/s)	Mildly reduced LV SF (41%). Restrictive pattern of diastolic filling (G3)	Moderate-Severe Reg (vAV Reg Vmax 346.72 cm/s)	Mildly dilated	Normal	51	Side by side great arteries, anterior aorta consistent with L Trasnpostion of the Great Arteries (congenitally corrected). S/P VSD repair with intact patch in basal septum
November 10, 2016	Transthoracic	Normal size and global systolic function (RV % FAC, A4C: 54.5%)	Moderate-Severe Reg (sAV Reg Vmax 5.28 m/s)	Mildly reduced LV SF (41%). Moderate hypokinesis of entire septal wall. Normal pattern of LV diastolic filling	Moderate Reg (vAV Reg Vmax 246.26 cm/s). Mildly thickened	Mildly dilated	Dilated	N/A	S/P BiV PPM
February 16, 2017	Transthoracic	Mildly reduced global RV SF (RV % FAC, A4C: 44.7%). RV wall thickness is moderately increased	Severe Reg (sAV Reg Vmax 5.00 m/s)	Mildly reduced LV SF (48%). Normal size	Mild-moderate Reg (vAV Reg Vmax 332.84 cm/s)	Moderately dilated	Normal	52	Interim mild improvement in systemic RV function but persistent systemic AV valve severe regurgitation with moderate pulmonary hypertension
February 27, 2017	Transthoracic	EF 54% (biplane)		EF 51% (A4C)					Limited study to quantify ventricular function
September 25, 2018	Transthoracic	Mild dysfunction. RV wall thickness is moderately increased	Moderate-Severe Reg (sAV Reg Vmax 4.74 m/s)	Mildly reduced LV SF (47%). Normal size	Mild Reg (vAV Reg Vmax 243.67 cm/s)	Mildly dilated	Normal	32	Overall no major changes noted compared to prior studies eccept for mild fluctuations in systemic RV function.
May 1, 2022	Transthoracic	Mildly reduced global RV SF (40%)	Moderate-Severe Reg (sAV Reg Vmax 4.71 m/s)	EF 45%	Moderate Reg (vAV Reg Vmax 328.85 cm/s)	Moderately dilated	Normal	46	There is a 1.3 cm × 1.3 cm, well circumscribed mass with echolucent center, seen apically, and likely represents a thrombus. Saline contrast bubble study -ve
May 4, 2022	Transesophageal	N/A	Moderate-Severe Reg (sAV Reg Vmax 2.46 m/s)	Moderately reduced LV SF (35%). Normal thickness	Moderate-Severe Reg (vAV Reg Vmax 383.31 cm/s)	N/A	N/A	N/A	Well-circumscribed mass measuring 2.07 cm × 1.43 cm in the morphologic RV/Systemic ventricle with central echolucency. Saline contrast bubble study was negative
September 30, 2022	Transthoracic	Mildly reduced global RV SF (40-45%). Mildly enlarged sRV	Mild-Mod Reg	Low-normal LV SF function	Moderate-Severe Reg (vAV Reg Vmax 228.43 cm/s)	Upper normal	Normal	24	Interim resolution of small systemic RV apical thrombus. Extensive trabeculation related to systemic RV hypertrophy noted

A4C: Apical 4 chamber; EF: Ejection fraction; FAC: Fractional area change; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; PPM: Permanent pacemaker; RA: Right atrium; RV: Right ventricle; SAV: Systemic atrioventricular valve; SF: Systolic function; S/P: Status post; SRV: Systemic right ventricle; vAV: Venous atrioventricular valve; vLV: Venous left ventricle; VSD: Ventricular septal defect; N/A: Not applicable.