Baylor University Medical Center Proceedings October 2017 - 437

Combined atresia of one left-sided and one right-sided
cardiac valve in a premature newborn
William C. Roberts, MD, Alan C. Sing, MD, and Joseph M. Guileyardo, MD

Described herein is the heart of a 2-day-old newborn, the product of a
25-week gestation, with atresia of two cardiac valves, one on the right
side and one on the left side, apparently a previously undescribed entity.

he worst heart disease-the one allowing the shortest
survival-is aortic valve atresia, the most common cause
of death in the ﬁrst month of life (1, 2). About 25% of
these newborns with aortic valve atresia also have mitral
valve atresia. The second most common condition associated
with an atretic cardiac valve is pulmonic valve atresia. The
occurrence of one right-sided atretic valve and one left-sided
atretic valve in the same heart must be incredibly rare, but such
was the case in the newborn described herein.
CASE DESCRIPTION
A 2-day-old female newborn after a 25-week gestation
weighed 550 g. An electrocardiogram shortly after birth disclosed a prolonged P-R interval and sinus bradycardia. A technically diﬃcult echocardiogram disclosed a dilated right ventricle,
a normal-sized left ventricle, a large atrial septal defect with
bidirectional ﬂow, a large ventricular septal defect with bidirectional ﬂow, a small (1 mm) patent ductus arteriosus with leftto-right ﬂow, severe "pulmonic stenosis," and an unobstructed
aortic valve and aortic arch. The echocardiographic ﬁndings
were interpreted as being consistent with tetralogy of Fallot.
The newborn died in the intensive care unit on the second
day of life. At necropsy, the heart weighed 4.15 g. The cardiac
ﬁndings are illustrated in the Figure. Both the mitral and pulmonic valves were atretic, the atrial septum was absent, and a
ventricular septal defect was located caudal to the aortic valve,
which arose from the dilated right ventricle. A narrowed patent ductus was present, and it was the only source of blood to
the lungs.
In addition to the cardiac anomalies, a cleft lip was present,
and it extended to involve the entire hard and soft palate.
Cytogenetic SNP microarray analysis performed on an antemortem blood sample disclosed a normal female chromosome
pattern with no deletions or duplications of known or potential clinical signiﬁcance. Postmortem chromosome analysis also
showed a normal female 46 XX karyotype. No numerical or
Proc (Bayl Univ Med Cent) 2017;30(4):437-438

Narrowed
patent
ductus
LMPA

Atretic
pulmonic
valve

Pulmonary
trunk

Absent
atrial
septum

SVC

Origin of
descending aorta
from right
ventricle

LA
RA
AA

Atretic
mitral
valve

IVC

Ventricular
septal
defect

Figure. Cardiac findings of a premature newborn with combined pulmonic and
mitral valve atresia. AA indicates ascending aorta; IVC, inferior vena cava; LA,
left atrium; LMPA, left main pulmonary artery; LV, left ventricle; RA, right atrium;
RV, right ventricle; SVC, superior vena cava.

From the Baylor Heart and Vascular Institute (Roberts) and the Departments of
Pathology (Roberts, Guileyardo) and Internal Medicine (Division of Cardiology)
(Roberts), Baylor University Medical Center at Dallas; and the Department of
Pediatric Cardiology, Texas Health Presbyterian Hospital, Dallas, Texas (Sing).
Corresponding author: William C. Roberts, MD, Baylor Heart and Vascular
Institute, 621 N. Hall Street, Suite H-030, Dallas, TX 75226 (e-mail: William.
Roberts1@bswhealth.org).
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Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings October 2017