Download Table 1 - Karger

Table 1. Comparison of clinical and full autopsy findings
Pt
Gender
GA
Death at
Side
Clinical cause of death
Full autopsy cause of death
Additional findings at full autopsy
left
Associated
anomalies/
morbidity
-
1
female
36+5
16
PPHN
hypotension
possible infection
lung abnormalities
1
left
-
PPHN
extensive lung abnormalities
38+2
10
left
-
PPHN
therapy-resistant hypotension
therapy-resistant PPHN
male
27+5
1
left
IRDS
PPHN
ARDS
(immature hypoplastic lungs)
female
38
6
left
aortic coarctation
(prenatal PLUG)
thrombo-embolic complications
and hemorrhage
thrombo-embolic complications
and hemorrhage
lungs:
- hypoplastic left lung (LHW)
- congestion
- extensive intra-alveolar hemorrhage
- extensive hyaline membranes
- signs of amniotic fluid inspiration
- stage: unable to determine
extramedullary hematopoiesis liver, heart, spleen
lungs:
- hypoplastic left lung (LHW)
- slight congestion
- hyaline membranes
- thickened wall of arterioles
- extramedullary hematopoiesis
- dilated lymph vessels at pleural surface
- signs of amniotic fluid and meconium aspiration
- stage: alveolar
kidneys: multiple tubular cysts
testes: cystic dysplasia
lungs:
- hypoplastic left lung (LHW)
- intra-alveolar hemorrhage
- early septal fibrosis
- thickened wall of arterioles, mainly left lung
- type II pneumocyte hyperplasia
- stage: unable to determine
kidneys: small asphyctic hemorrhages
lungs:
- hypoplastic lungs (LHR)
- intra-alveolar and interstitial hemorrhage
- hyaline membranes grade III
- thickened wall of arterioles
- stage: canalicular
heart: perimembraneous VSD, bicuspid pulmonary valve
kidneys: multiple small tubular cysts
liver: ductal plate formation
lungs:
- hypoplastic lungs (LHW)
2*
male
34+3
3
male
4*
5
- congestion
- extensive septal and intra-alveolar hemorrhage
- fibrotic aspect
- multiple thrombi arteries/arterioles
- stage: saccular
heart: several thrombi; several infarctions due to thrombi; open ductus;
coarctatio aorta
liver: extensive congestion, cholestasis, zone 3 necrosis
kidneys: extensive hemorrhage and congested left kidney
* denotes this patient did not receive ECMO treatment.
Pt = patient anonymous code GA = gestational age at birth (in weeks
+ days
) Death at = time of death in postnatal days Side = side of CDH LHW = lung hypoplasia based on
lung weight versus GA normal values LHR = lung hypoplasia based on lung weight : body weight-ratio (< 0.012) PPHN = persistent pulmonary hypertension of the neonate
IRDS = infant respiratory distress syndrome ARDS = acute respiratory distress syndrome VSD = ventricular septal defect
Supplementary Table 2. Comparison of clinical and lung biopsy findings
Pt
Gender
GA
Death at
Side
Assoc Anomalies/
Morbidity
Clinical cause of death
Additional findings at lung biopsy
1
Female
38+6
11
right
hemivertebra T12
PPHN
congestion
intra-alveolar and interstitial hemorrhage
hyaline membranes
thickened wall of arterioles
thrombi
stage: unable to determine
2
Male
37+6
18
left
-
PPHN
(fibroblast isolation)
congestion
intra-alveolar hemorrhage
hyaline membranes, mild septal fibrosis
thickened wall of arterioles
extramedullary hematopoesis
local bronchopneumonia
stage: saccular
3
Female
39+6
12
left
-
PPHN
congestion
intra-alveolar hemorrhage
thickened wall of arterioles
slight extramedullary hematopoesis
stage: saccular
4
Female
36+5
19
left
pneumothorax, small
ependymal hemorrhage
PPHN
congestion
hemorrhage
hyaline membranes, septal fibrosis
thickened wall of arterioles
extramedullary hematopoesis
atelectasis
type II pneumocyte hyperplasia
stage: unable to determine
5
Female
38
14
left
sick euthyroid syndrome
PPHN
congestion
intra-alveolar hemorrhage
thickened alveolar septa
thickened wall of arterioles
extramedullary hematopoesis
atelectasis
type II pneumocyte hyperplasia
stage: saccular
6
Male
37
23
left
-
PPHN + MOF
extensive hemorrhage
thickened alveolar septa
thickened wall of arterioles
thrombi
centroacinary unfolding with many atelectasis
type II pneumocyte hyperplasia
stage: saccular-alveolar
7
Female
38
12
right
-
Right-sided heart failure
due to PPHN
extensive intra-alveolar hemorrhage
slight fibrosis
thickened wall of arterioles
atelectasis
type II pneumocyte hyperplasia
stage: saccular
8
Female
38+1
29
left
-
PPHN
congestion
extensive hemorrhage
start of hyaline membranes, septal fibrosis
thickened wall of arterioles
extensive type II pneumocyte hyperplasia
stage: saccular
9
Male
39+3
12
left
intracerebral
hemorrhage
PPHN
congestion
extensive intra-alveolar hemorrhage
thickened wall of arterioles
extramedullary hematopoesis
thrombi
partial atelectasis
stage: unable to determine
10
Male
39
16
left
prenatal cerebral
infarction
PPHN
thickened alveolar septa
thickened wall of arterioles
centroacinary unfolding
stage: alveolar
11
Female
41+1
20
left
pneumothorax
PPHN
12* Female
36+6
1
left
severe left ventricle
heart hypoplasia, mitral
atresia, large ASD,
double outlet right
ventricle
PPHN
intra-alveolar hemorrhage
thickened alveolar septa
thickened wall of arterioles
partial atelectasis
type II pneumocyte hyperplasia
stage: unable to determine
congestion
extensive hemorrhage
extensive hyaline membranes
thickened wall of arterioles
thrombi
centroacinar unfolding
stage: saccular
13* Male
36+2
2
left
Cornelia de Lange
syndrome, hypospadias
PPHN
minimal hemorrhage
thickened wall of arterioles
extensive atelectasis
amniotic fluid and meconium aspiration
stage: alveolar
14
Female
39+2
16
left
-
PPHN
intra-alveolar hemorrhage
hyaline membranes
thickened wall of arterioles
arterial thrombus
atelectasis
type II pneumocyte hyperplasia
stage: saccular
15
Female
38+3
21
left
pneumothorax
PPHN
congestion
hemorrhages
slight hyaline membranes, interstitial fibrosis
thickened wall of arterioles
atelectasis
stage: unable to determine
* denotes this patient did not receive ECMO treatment.
Pt = patient anonymous code GA = gestational age at birth (in weeks
+ days
) Death at = time of death in postnatal days Side = side of CDH Assoc anomalies = associated
anomalies PPHN = persistent pulmonary hypertension of the neonate ASD = atrial septal defect MOF = multi-organ failure