Journal of the Saudi Heart Association (2011) 23, 45–47

King Saud University

Journal of the Saudi Heart Association

www.ksu.edu.sa
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SHORT COMMUNICATION

Unusual cause of neonatal cyanosis

M.Y. Abd El Rahman *, M.M. Al Qurashi, F.A. Al Khalifeh

Division of Cardiology, Department of Pediatrics, Al Yamamah Hospital – Riyadh, Saudi Arabia

Received 11 April 2010; revised 17 July 2010; accepted 15 September 2010

Available online 14 October 2010

KEYWORDS Abstract We present a case of a full-term female neonate who presented at 6 h of age with severe
Cyanosis; cyanosis and was partially responsive to oxygen supplementation. An echocardiogram showed an
Newborn; isolated congenital severe tricuspid valve insufficiency due to rupture of the papillary muscle of
Rupture of the papillary the anterior tricuspid valve leaflet. Magnesium sulfate was infused to lower the pulmonary resis-
muscle; tance and thus enhancing the antegrade pulmonary blood flow. Ductal patency was secured by
Tricuspid valve prostaglandin infusion thus providing an additional pulmonary blood flow through the ductus arte-
riosus.
The above measures were adequate to stabilize the patient with no further deterioration or the
need for other supportive measures such as Nitric Oxide therapy or extracorporeal membrane oxy-
genation (ECMO). Therefore, early diagnosis and adequate measures to improve the pulmonary
blood flow are mandatory, important pre-operative measures in the management of these patients.
ª 2010 King Saud University. Production and hosting by Elsevier B.V. All rights reserved.

1. Introduction 2. Case report

Tricuspid valve regurgitation (TR) due to chordal rupture is an
extremely rare differential diagnosis of neonatal cyanosis
(Riede et al., 2010).
* Corresponding author. Tel.: +966 14914444x1346; fax: +966
1208360.
E-mail address: moh-rahman1968@hotmail.com (M.Y. Abd El
Rahman).
1016-7315 ª 2010 King Saud University. Production and hosting by
Elsevier B.V. All rights reserved.
Peer review under responsibility of King Saud University.
doi:10.1016/j.jsha.2010.09.002
Production and hosting by Elsevier
A full-term female neonate was delivered at 38 weeks gestation
by normal spontaneous vertex delivery and weighed 3900 g at
birth. Patient’s APGAR scores were 9, 10 at 1 and 5 min,
respectively. Meconium staining was noticed during neonatal
resuscitation. Pregnancy was uneventful and pre-natal ultra-
sound was normal at 22 weeks of gestation. The mother did
not notice any decrease in the fetal movements during the last
trimester. She did not take drugs during the last trimester of
pregnancy even including prostaglandin synthetase inhibitors
and she did not have a history suggestive of systemic lupus
erythematosus.
Six hours after birth the neonatology team noticed severe
cyanosis and abdominal distention. The initial diagnosis was
meconium aspiration syndrome. However, patient cyanosis
did not improve by 100% oxygen supplementation.
Prompt cardiology consultation was made to rule out con-
genital heart disease. Cardio-vascular examination revealed a
46
distressed, cyanosed, nondysmorphic newborn. The heart rate
was 140/min, blood pressure was 66/34 mm Hg and the respira-
tory rate was 46/min. The initial oxygen saturation was 70%
which increased to 80% with 100% oxygen inhalation via head-
box. The capillary refill time was about 3 s and peripheral
pulses were equally felt. The liver edge was palpated 4 cm below
the right costal margin and the liver itself was abnormally pul-
sating. Sacral edema and ascites were present. The precordium
was hyperactive. Cardiac auscultation revealed normal first and
second sounds, no gallop rhythm and a harsh pan-systolic mur-
mur of grade 3/6 was heard at the left lower sternal border.
Chest X-ray revealed oligemic lung fields and there was
cardiomegaly.
Twelve leads standard ECG was performed and revealed a
sinus rhythm at a rate of 160/min, left axis deviation, promi-
nent P wave suggestive of P-pulmonale, no evidence of left
or right ventricular hypertrophy. There were no ST segment
changes, and the corrected QT interval was within normal lim-
its. Cardiac echocardiography using Vivid 7 ultrasound system
(GE, Horten, Norway) revealed normal segmental anatomy.
The right atrium was significantly dilated. A right to left shunt
was detected through a stretched patent foramen ovale. The
right ventricle was hypertrophied. The diagnosis of rupture
of the tricuspid valve (TV) papillary muscle was made after
identification of flail antero-superior leaflet of the TV with a
thickened echogenic tip and the absence of a connection be-
tween the anterior papillary muscle and the flail leaflet of TV
(Fig. 1). The tip of the anterior papillary muscle appears echo
bright. Severe TV regurgitation was present (Fig. 2) and the
tricuspid valve regurgitation pressure gradient was 55 mm Hg.
The antegrade pulmonary flow was reduced in the color Dopp-
ler mode, however, no detectable pressure gradient across the
pulmonary valve was elicited. The ductus arteriosus was closed
and no area of calcification was present in the usual ductal
location. The right atria was dilated and right to left shunt
was observed through a stretched patent foramen ovale
(PFO). There were no vegetations or thrombi. CK and CK
MB enzymes were within normal limits. No laboratory evi-
dence of maternal lupus was found.
The patient was promptly stabilized by inotropes, prosta-
glandin E1, magnesium sulfate and Lasix therapy. 100%
Figure 1 Apical four-chamber image in two dimensional Echo
during systole demonstrating the flail anterior tricuspid valve (TV)
leaflet with echo-bright density. Abbreviations: RA, Right atrium;
RV, Right ventricle; LA, Left atrium; LV, Left ventricle; TV,
Tricuspid valve leaflet; MV, Mitral valve.
M.Y. Abd El Rahman et al.
Figure 2 Apical four-chamber image with color Doppler flow
during systole showing the severe degree tricuspid regurgitation
(TR). Abbreviations: RA, Right atrium; RV, Right ventricle; TR,
Tricuspid regurgitation; LA, Left atrium; LV, Left ventricle.
oxygen (7 l/min) inhalation by headbox was sufficient to main-
tain her oxygen saturation at 90%. The above measures were
adequate to stabilize the patient with no further deterioration
or need for other supportive measures such as Nitric Oxide
therapy or ECMO.
Repeated examination at 24 h after birth revealed improved
patient cyanosis which allowed the gradual withdrawal of oxy-
gen therapy and inotropic support. Repeated echocardiogra-
phy at the age of 2 weeks confirmed the diagnosis. The shunt
across the patent foramen ovale (PFO) became mostly left to
right. Sufficient antegrade and minimal retrograde pulmonary
blood flow was seen. Surgical intervention will be planned elec-
tively in the near future.
3. Discussion
Neonatal tricuspid valve insufficiency frequently complicates
other forms of congenital heart disease (Kobza et al., 2004;
Kanter et al., 2004). In contrast there are few case reports in
the literatures regarding neonatal critical tricuspid regurgita-
tion due to ruptured papillary muscle or chordae (Anagnosto-
poulos et al., 2007 Apr; Katogi et al., 1998).
Most of the reported cases as well as the present case pre-
sented with congestive heart failure and cyanosis (Lim et al.,
2004). The tricuspid regurgitation in these cases may be severe
enough to preclude antegrade pulmonary flow, with a resultant
massive right to left atrial shunt (Lim et al., 2004).
The differential diagnosis of papillary muscle rupture can
be broadly divided into ischemic and non-ischemic etiologies
(Fleming et al., 2008).
Non-ischemic causes include trauma and infective endocar-
ditis (Fleming et al., 2008). Both can be ruled out in our case
since the delivery was uneventful and there was no clinical or
laboratory findings consistent with the infection. In addition
there was no evidence of any vegetation in the immediate
post-delivery echocardiogram.
Myocardial ischemia may result from antepartum or peri-
partum asphyxia (Riede et al., 2010), viral infections (Marton
et al., 2002; Lazda et al., 2000), Rhesus isoimmunisation
(Marton et al., 2002) and maternal auto-immune disease
Unusual cause of neonatal cyanosis
(Fleming et al., 2008). The papillary muscles are particularly
vulnerable to ischemia because they lie at the distal extremes
of coronary circulation and require a greater oxygen supply
than subepicardial myocardium (De Busk and Harrison, 1969).
In the present case, the delivery was carried out smoothly,
patient’s Apgar score was acceptable and patient’s cardiac en-
zymes were within the normal range thus eliminating the pos-
sibility of perinatal ischemia.
Although the cause of papillary rupture is unclear in our
case, however, one might speculate on transient ischemia dur-
ing the pregnancy. The echo bright flail appearance of anterior
tricuspid valve leaflet (Fig. 1) suggests in utero insult rather
than an acute perinatal insult (Sachdeva et al., 2007). Antena-
tal ductal closure is of interest beyond its importance in
limiting treatment options. If closure occurred suddenly, a tre-
mendous increase in right ventricular afterload would result
during severe fetal stress, and the combined hemodynamic
and metabolic insult might provoke ischemic rupture of the tri-
cuspid papillary muscle (Sachdeva et al., 2007). However, there
was no history of late gestational intake of prostaglandin syn-
thetase inhibitors (PSI) in our patient to explain the premature
ductal closure. Chronic in utero ductal closure is unlikely in
the present case because no area of calcification was present
in the usual ductal location.
The marked cyanosis observed immediately after birth is
due to marked right to left shunt at the atrial level due to high
pulmonary resistance. MgSO4 infusion was given in the pres-
ent case to lower the pulmonary resistance and thus reducing
right to left shunt at the atrial level as well as to enhance the
antegrade pulmonary blood flow. Ductal patency was secured
by Prostaglandin E1 infusion thus providing an additional ret-
rograde pulmonary blood flow through the ductus arteriosus.
Such prompt management was successful in improving pa-
tient’s cyanosis. This signifies the importance of the time factor
in initiation of these simple measures to avoid the expense of
Nitric Oxide and complication of ECMO.
Acknowledgment
We are grateful to Maryam Al Mutary for providing the liter-
atures required for this case report.
47
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