Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome
Mio Taketazu, MD*,
Catherine Barrea, MD*,
Jeffrey F. Smallhorn, MD*,
Gregory J. Wilson, MD and
Lisa K. Hornberger, MD* ,*
* Fetal Cardiac Program, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Cardiovascular Research, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

View larger version (96K):
[in a new window]
|
Figure 1 (a) The normal fetal pulmonary vein Doppler spectra is composed of forward flow in ventricular systole (S) and diastole (D), with cessation of flow during atrial systole. (b) Reverse flow during atrial contraction in PV spectra is commonly shown in fetal left heart obstruction. Measurement of velocity-time integral for forward (VTIF) and reverse (VTIR) flow is demonstrated.
|
|

View larger version (77K):
[in a new window]
|
Figure 2 Three pulmonary vein flow patterns identified in fetuses with left heart obstruction. (A) Continuous forward flow with a small a-wave reversal (VTIR/VTIF ratio <0.18). (B) Continuous forward flow with an increased a-wave reversal (VTIR/VTIF ratio 0.18). (C) To-and-fro flow pattern with absent early diastolic forward flow. VTIF = velocity-time integral for forward flow; VTIR = velocity-time integral for reverse flow.
|
|

View larger version (14K):
[in a new window]
|
Figure 3 The ratio of pulmonary vein (PV) to pulmonary artery (PA) diameter compared between the three PV flow pattern groups. The PV/PA ratio in fetuses with type C flow was significantly increased compared with that in fetuses with type A or B flow. The mean PV/PA ratios were 0.76 ± 0.19 in type A, 0.73 ± 0.23 in type B, and 1.53 ± 0.24 in type C flow.
|
|

View larger version (21K):
[in a new window]
|
Figure 4 Outcome of patients with perinatal surgical intervention. Perioperative survival was 91% in patients with type A, 100% with type B, and 33% with type C pulmonary vein flow. HTX = heart transplant.
|
|

View larger version (118K):
[in a new window]
|
Figure 5 Histologic findings of the lung tissue in one patient with a prenatal diagnosis of hypoplastic left heart syndrome and a type C pulmonary vein (PV) flow pattern, who died at seven weeks of age. (a) There were dilated lymphatic vessels (L) and PVs with internal and external elastic lamellae, consistent with early stage arterialization. (Movat's stain, x100 magnification.) (b) Muscular pulmonary artery with medial (M) thickening and neointimal (N) hyperplasia, indicators of pulmonary hypertension, Heath-Edwards grade II, in the same infant. (Movat's stain, x400 magnification.)
|
|
|