Follow-up
Median follow up time after correction was 19 years (range 1 to 27
years). Overall survival after definitive correction was 96% at 20
years (Figure 2). One patient died four years after successful
correction of unknown cause, probably of cardiac arrhythmia. Two
patients died 20 years after correction due to progressive heart
failure. Two of these had 22q11 deletion. From the survivors all but one
are in NYHA class I or II. Four of the seven patients who were not
suitable for correction died. One patient died 2 years after the last
unifocalization due to respiratory failure and infection. One patient
died of unknown cause 9 years after the last unifocalization procedure.
One patient died 14 years after the last unifocalization of multi-organ
failure and sepsis and one patient died of massive intracranial bleeding
9 years after the last unifocalization.
Among the 5 survivors with 22q11 deletion, one is awaiting correction,
one is palliated in a reasonable condition, 3 had a definitive
correction (one in reasonable condition and 2 in good condition). The
overall survival in the 22q11 deletion patient was significantly
(p=0.041) lower compared to non-syndromic patients, (5 out of 10 (50%))
versus 25 out of 29 (67%) respectively).
Pulmonary valve replacement
After final correction in 21 patients other interventions were
performed. They are listed in Table 1 and consist mostly of pulmonary
valve replacement either surgically or percutaneously and dilatation or
stenting of pulmonary branches. The modified Blalock Taussig shunt
mentioned in the Table was placed in a patient with stenosis of a
hypoplastic left pulmonary artery. Freedom from pulmonary valve
replacement was 88%, 73%, 60% and 27%% at 5, 10, 15 and 20 years
respectively (Figure 3).
Echocardiography
Echocardiographic data after correction at the last check, showed in 18
patients (75%) a reasonable or good right ventricular function (RVF).
Four patients (17%) had a moderate RVF. Only 2 patients (8%) had a
severely impaired RVF.
The tricuspid regurgitation was trivial, mild, and moderate in 10
(42%), 10 (42%) and 4 patients (16%), respectively. The pulmonary
regurgitation was absent/trivial, mild, moderate or severe in 6 (26%),
8 (35%), 6 (26%) and 3 (13%), respectively. The right ventricular
dilatation was absent, mild, moderate and severe in 2 (8%), 4(17%), 12
(50%) and 6 (25%), respectively. If measurable, the median right
ventricular (RV) pressure was estimated at median of 54 (25-108) mmHg.
The median estimated pressure across the homograft is 19 (7-49) mmHg.
Inherent thereto, the calculated pressure differences of 32 (0-95) mmHg
suggests increased pulmonary artery pressures.
In 7 patients a small residual VSD was present, without hemodynamic
significance in terms of flow.
MR imaging
From 17 patients after correction we obtained detailed MR imaging with a
median interval between correction and MR image of 15,6 years (range
9-22 years). Based on the calculations the median right ventricular
ejection fraction (RVEF) was 44% (range 13-62%), the median left
ventricular ejection fraction (LVEF) was 52% (range 29-64%), the
median RV end diastolic volume was 190 ml (range 94-339 ml), indexed 105
ml/m2 (range 76-176 ml/m2) and the median pulmonary regurgitation
fraction was 19% (range 0-50%).