Download Congestive Heart Failure and Absent Femoral Pulses in Newborns

Congestive Heart Failure and
Absent Femoral Pulses in
Newborns without Coarctation of
the Aorta
Cuillermo R. Sdnchez, M.D.;* Charles D. Vinocur, M.D.;*
Charles U! Wagner, M.D.;* Glenn S. Kaplan, M.D.;*
S. David Rubenstein, M. D.;* Fre&rickJ. Fricker, M. D.;t and
Yoshie Hashida, M. D . t
Two infants with thrombosis of the abdominal aorta are
discussed. In each case the presentation was indistinguishable from that in coarctation of the aorta, with heart failure
and absent femoral pulses. Surgery in one infant successfully relieved the obstruction. The diagnosis may not be
suspected from the history. Aggressive management is
indicated.
v
ascular occlusion in the newborn is an uncommon
clinicopathologic entity associated with significant morbidity and mortality.' Several reports have described the
development of aortic thrombosis in infants with a history of
umbilical artery catheterization and have demonstrated the
high mortality associated with this c o m p l i c a t i ~ nWe
. ~ ~present two additional cases of aortic occlusion in infancy,
emphasizing the clinical presentation as being indistinguishable from that of severe coarctation of the aorta, and
demonstrating that aggressive therapy may be successful.
infusion were begun, but the infant showed little improvement, and
cardiac catheterization with angiography was performed. The study
showed a normal aortic arch, a patent ductus arteriosus, and no
coarctation. Flow in the descendingaorta was sluggish, and the distal
aorta was not visualized. The impression was thrombotic occlusion of
the abdominal aorta. Despite heparinization and intensive medical
efforts, the infant died 19 hours after admission.
At autopsy a large thombus was found occluding the abdominal
aorta from a point just proximal to the renal arteries to the bifurcation
of the aorta (Fig 1). The thrombus propagated into the left renal
artery, and small recent infarcts were present in the left kidney. No
inflammation or degeneration was noted in the adjacent aortic wall,
and no venous thrombii were present.
A 3,700-g male was born at term to a mother with gestational
diabetes mellitus. Vaginal delivery was complicated by shoulder
dystocia, with asphyxia and Apgar scores of 1and 1 at one and five
minutes, respectively. Umbilical artery and vein catheters were
inserted, bicarbonate was administered (exact route not known), and
the infant was transferred to another hospital. The hemoglobin prior
CASE1
An eight-day-old male had been born by cesarean section at term
to a mother with gestational diabetes mellitus. Birth weight was
3,300 g, and Apgar scores were 4 and 8 at one and five minutes,
respectively. He was meconium stained, but was not intubated, and
the umbilical vessels were not catheterized. He was discharged at
five days ofage. Two days later respiratory distress developed, and he
was transferred to Children; Hospital of Pittsburgh. The systolic
pressure was 56 mm Hg in the ri&t arm and 30 m i Hg in &e left
leg. there was no murmur, the liver was enlarged, and the femoral
pulses were absent. Chest roentgenogram showed cardiomegaly and
increased vascular markings. The ECG was normal. M-mode
echocardiogram showed diminished left ventricular shortening fraction. Hemoglobin was 16.6 g/dl and hematocrit 51.5 percent; the
~ with polymorphonuclear preponWBC count was 1 2 . 7 0 0 1 ~mm,
derance; the platelets were 120,00O/cu mm; PT was 12 seconds
(control, 11.5 seconds), and PIT 36 seconds (control, 34 seconds).
The clinical impression was coarctation of the aorta and congestive
heart failure. Mechanical ventilation, digoxin, and prostaglandin E,
*Departments of Pediatrics and Surgery, St. Christopheri Hos ital
for Children and Temple University School of Medicine, ~ f i l a delphia.
?Departments of Pediatrics and Pathology. Children's Hospital of
Pittsburgh and the University of Pittsburgh
- School of Medicine,
~ittsbura.
Reprint requests: D1: Sdnchez, Division of Pediatric Cardiology, St.
Christopheri Hospital, Philadelphia 19133
I
.
.....,.. ..... ....--
..1romFIGURE
. ..v.vg.mtr..
.,. -u.uy.,
rtrrr
-...,
bus originating at the level of the renal arteries and occluding the
distal abdominal aorta. The umbilical arteries remain connected to
the umbilicus stump.
CHF in Newborns ~ i t h t WCOE~clation
l
(Sanchez eta)
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21351/ on 05/13/2017
to transfer was 12.9 gldl. In the first day of life, the left leg became
cyanotic, but this cleared after immediate removal of the catheter.
Seizureswere controlled with phenobarbitaland phenytoin, and the
infant's status improved until the fifth day of life, when both legs
became transiently cyanotic and the femoral pulses diminished.
During the subsequent four days, the femoral pulses became more
diacult to appreciate, and on the tenth day of life, signs ofcongestive
heart failure and pulmonary hemorrhage developed. Digoxin and
antibiotic therapy, and mechanical ventilation were begun, and the
infant was transferred to St. Christopher's Hospital for Children. On
examination, the infant was hypertonic with occasional spontaneous
respirations and frequent seizure activity. Systolic pressure was 140
mm Hg in the left arm and 50 mm Hg in both legs. Small amounts of
blood were suctioned from the endotracheal tube. A continuous
murmur was present at the left infraclavicular space, the liver was
enlarged, and the femoral pulses were absent. The ECG showed
biventricular hypertrophy, and a roentgenogram of the chest showed
cardiomegaly, with parenchymal haziness and scattered densities
compatible with congestive heart failure and pulmonary hemorrhage. A two-dimensional echocardiogram failed to satisfactorily
visualize the distal aortic arch. Hemoglobin was 13.6 g/dl and
hematocrit, 40.8 percent. The white blood cell count was 14,0001~~
mm, with polymorphonuclear preponderance; and platelets,
58,0001~~
mm. There was occult hematuria. An infusion of prostaglandin E, was begun, but six hours later the infant's status was not
improved, and cardiac catheterization with angiography was performed. The study showed a normal aortic arch and complete
occlusion of the distal abdominal aorta proximal to the ostia of the
renal arteries (Fig2).Thmmbolytictherapy was not initiated because
d b d a M&rr
h i s . but in view of hwertension and CNS
FIGURE
2. Angiogram m me aescenalng aorta in the posteroanterior
projection showing complete occlusion of the distal abdominal aorta
beyond the origin of the superior mesenteric artery. The renal
arteries do not opacify.
irritability, surgical embolectomy was attempted via a transverse
lower abdominalincision and transperitonealapproach. The abdominal aorta was opened just above the level of the bifurcation, and a
large thrombus was removed. Smaller thrombi were also removed
from both iliac arteries with Fogarty catheters. There was prompt
resolution of the systemic hypertension, and appearanceofexcellent
pulses in the lower extremities. Heparin therapy was begun eight
hours after surgery and was continued for three days. Neurologic
problems persisted which required adjustment of medications.
Seven weeks after surgery, the infant was discharged with normal
blood pressure and pulses.
Thrombotic occlusion of the abdominal aorta in infants is
rare, and has been observed in association with umbilical
artery ~atheterization,'~as well as in cases where the
umbilical vessels had not been m a n i p ~ l a t e d . ' . ~Sepsis,
'~
polycythemia, maternal diabetes, dehydration, and clotting
factor abnormalities have been considered to play a role in
the etiology.'."," In those patients with no history of umbilical artery catheterization, it has been suggested that thromboembolic phenomena may b e related to the changes in the
circulation at birth,'.u with emboli originating in a patent
ductus arterios~s'.'."'~." or in the umbilical vein."
Our two patients illustrate the typical presentation and
rapid deterioration characteristic of this disorder. The nature
of the problem in case 1 was unsuspected, while in case 2 it
was suspected from the history. In both cases the role of
maternal diabetes and perinatal asphyxia in the development
of aortic thrombosis must remain speculative, but in case 2
the umbilical artery catheter and hypertonic fluid infusions
must b e considered contributing hctors. Polycythemia was
not present in either infant. Although in our cases the
diagnosis was confirmed by angiography, other investigators
have used Doppler blood flow and echocardiography as well
as radionuclide perfusion studies6 to diagnose the condition
noninvasively, thus avoiding the use of angiographic contrast
media that could decrease the chances of survival.
Although conservative management has proven successful
in cases of peripheral occlusive disorders in infants,',' we are
not aware of survival of any infant with symptomatic occlusion of the abdominal aorta with medical therapy alone. It is
possible that thrombolytic
may provide an alternative to the management of these critically ill infants, but
success may depend on early recognition and diagnosis. It is
questionable whether thrombolytic therapy could have been
successfully administered in case 2 in view of signs of
pulmonary hemorrhage. The value of postoperative anticoagulation or thrombolytic therapy remains to b e defined.
The clinical course of these two infants demonstrates that
thromboembolic aortic occlusion must b e suspected in any
infant who presents with signs suggestive ofcoarctation ofthe
aorta, and that aggressive surgical management of the symptomatic infant can successfully relieve the obstruction.
Since submitting this manuscript, a report has appeared"
describing the failure of intra-arterial thrombolytic therapy
to induce clot lysis in a newborn with aortic occlusion.
Failure was attributed to inability to induce a hyperfibrinolytic state due to physiologic reduction in plasminogen
levels.
CHEST 1 83 I 4 1 Aprll, 1983
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21351/ on 05/13/2017
688
ACKNOWLEDGMENT: We wish to acknowledge the assistance
provided by Marianna Donleavy in the preparation of the manuscript
REFERENCES
1 Gross RE. Arterial embolism and thrombosis in infancy. Am J
Dis Child 1945; 70:61-73
2 Anderson VM, Harris PJ. Thrombotic occlusion of the aorta in
infants of diabetic mothers [abstract]. Pediatric Pathology Club
Fall Meeting, Providence, RI. Oct 11-13, 1979.
3 Henry CG, Gutierrez F, Lee JT, Hartmann AF, Bell MJ, Bower
RJ. et al. Aortic thrombosis presenting as congestive heart
failure: An umbilical artery catheter complication. J Pediatr 1981;
98:820-22
4 Doshi U, Bhat R, Rao S, Flanigan D, Vidyasagar D. Neonatal
aortic thrombosis [Letter]. J Pediatr 1981; 99:1002-03
5 Bhat R, Fisher E, Doshi U, Roza S, Sepehri B, Flanigan P, et al.
Neonatal abdominal aortic thrombosis. Crit Care Med 1981;
9:858-61
6 O'Neill JA, 'Neblett WW 111, Born ML. Management of major
thromboembolic complications of umbilical artery catheters. J
Pediatr Surg 1981; 16:972-78
7 Stout C, Koehl G. Aortic embolism in anewborn infant. Am J Dis
Child 1970; 120:74-76
8 Ordway NK, Stout LC. Thromboembolus to abdominal aorta in a
neonate. J Pediatr 1973; 82:143-48
9 Bjarke B, Herin P, Blomback M. Neonatal aortic thrombosis: a
possible clinical manifestation of congenital antithrombin 111
deficiency. Acta Paediatr Scand 1974; 63:297-301
10 Knowlson GT, Marsden HB. Aortic thrombosis in the newborn
period. Arch Dis Child 1978; 53164-66
11 Oppenheimer EH, Avery ME. Clinical pathological conference.
J Pediatr 1968; 73:143-49
12 Henriksson P, Wesstrom G, Hedner U. Umbilical artery
catheterization in newborns: 111. Thrombosis-a study of some
~ r e d i s ~ o s i nfactors.
g
Acta Paediatr Scand 1979; 68:719-23
13 Woodward JR, Patterson JH, Brinsfield D. Renal artery tbrombosis in newborn infants. Am J Dis Child 1967; 114:191-94
14 McFaul RC, Keane JF, Nowicki ER, Castaneda AR. Aortic
thrombosis in the neonate. J Thorac Cardiovasc Surg 1981;
81:334-37
15 Delaplane D, Scott JP, Riggs TW, Silverman BL, Hunt CE,
Urokinase therapy for a catheter-related right atrial thrombus. J
Pediatr 1982; 100:149-52
16 Corrigan JJ Jr, Allen HD, Jeter M, Malone JM. Aortic thrombosis in a neonate: failure of urokinase thrombolytic therapy. Am
J Pediatr Hematol Oncol 1982; 4:243-47
Paroxysmal Nodal Reentrant
Tachycardia*
Surgical Cure with Preservation of
Atrioventricular Conduction
Joaquin Marquez-Montes, M.D.;Juan J. Rufilanchas, M . D . ;
Juan J. Esteve, M . D . ; Lour&s Alvarez, M . D . ;
Juan Benezet, M . D . ; Raul Burgos, M . D . ; and
Diego Figuera, M . D . , EC.C.I!
*From the Division of Cardiology and Cardiovascular Sur e
Centro Nacional de Especialidades Medie~Quirurgicasf e y ;
Seguridad Social, Clinica Puerta de Hierro (Universitary Hospital),
Madrid, Spain.
690
In a patient with repetitive disabling tachycardias refractory to pharmacologic treatment, t h e electrophysiologic
study suggested t h e existence of atrioventricular nodal
reciprocating tachycardia. D u r i n g ventricular pacing, e n doepicardial mapping of t h e lower atrium showed t h e atrial
breakthrough point in a n a r e a of t h e lower interatrial
septum close to t h e AV node crista. A selective atriotomy was
performed. T h e postoperative electrophysiologic studies
showed absence of ventriculoatrial conduction a t several
ventricular pacing rates, while antegrade conduction is
preserved. f i e patient remained free of arrhythmias 21
months after surgery, taking n o antiarrhythmic drugs.
ectrophysiologic studies using programmed stimulaE l tion,14 endoepicardial m a p p i n g , ' . h n d surgical results"' have led to a better knowledge of AV junctional
reciprocating tachycardias. There is more experience in t h e
surgical treatment of AV junctional reciprocating tachycardias incorporating Kent bundles in t h e circuit. However,
those tachycardias with intranodal mechanism o r those
incorporating other types of accessory pathways are less wellknown, and surgical treatment experience is limited.&"
T h e surgery in the latter type ofAV reciprocating tachycardias basically d e p e n d s o n t h e surgical interruption o r
cryoablation of t h e His bundle, as demonstrated by Harrison
e t a18 and Klein e t al.' Pritchett e t all0 and Ward e t al" have
recently reported surgical correction of arrhythmia while
preserving AV conduction.
We recently operated o n a patient with recurrent AV
reciprocating tachycardia which was refractory to medical
treatment, using endoepicardial mapping of t h e lower atrium
close to the AV junction prior to AV interruption of t h e
tachycardia circuit. T h e patient remained free of arrhythmias
21 months after surgery, with preserved AV conduction.
The patient was a 62-year-old woman with no clinical or hemodynamic evidence of heart disease, who had had bouts ofpalpitations
since 1966. Initially, the tachycardia appeared once or twice a month,
lasted for 15 to 30 minutes, and remitted spontaneously. Since 1975
the tachycardia episodes had become more frequent (five to six per
month) and lasted longer. From 1979on, the crises increased to three
or four per week, rangingfrom three to 12 hours, with occasional syncope. Furthermore, the patient showed evidence of severe impairment of her quality of life and was unable to perform her usual
activities.
The arrhythmia remitted temporarily after intravenous (IV) administration of verapamil (10 mg), ajmaline (1 mglkg), or mexiletine
(250 mg as single doses). Multiple oral antiarrhythmic drugs were
used without preventing tachycardia, the only ones showing some
effect being arniodarone (600mg/day)and quinidine sulfate (1g/day),
or the association of digoxin (0.25 mg/day) with verapamil (240 mg/
day), which lengthened the intercrisis interval and shortened the
crises.
Preoperative Electrophysiologic Study
This study was camed out to assess the mechanism of arrhythmia
and the action antiarrhythmic drugs and diverse modalities ofcardiac
pacing.
The basal ECG showed sinus rhythm with 0.12-sec P-R interval,
left anterior hemiblock and occasionally right bundle branch block
(Fig LA). All antiarrhythmic medication was suspended one week
before the study. Three multipolar electrode catheters were inserted
Paroxysmal Nodal Reentrant Tachycardia (Marquez-Monteseta/)
Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21351/ on 05/13/2017