Author
Information
Monali
Dudhe*, Neha Saxena*, Rashmi Khadkikar**, Shabnam Qureshi**, A. R. Chauhan***
(*
Third Year Resident, ** Assistant Professor, *** Additional Professor.
Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital ,
Mumbai ,
India
Abstract
Coarctation of aorta (stenosis commonly located at the junction of the
arch of aorta and proximal descending aorta) may be diagnosed for the first
time during pregnancy, as most patients are asymptomatic. Dilatation and
dissection of the aorta can lead to increased maternal mortality; significant
stenosis is a contraindication to pregnancy. However, successful pregnancies
have been reported in women with uncorrected coarctation if preconception risk
stratification is done. We report a primigravida who presented with chronic
hypertension and breathlessness where the diagnosis of coarctation was made
incidentally during active labor in view of differences in blood pressure (BP)
readings of both arms. She underwent emergency lower segment cesarean section
(LSCS) and remained stable postoperatively.
Introduction
Coarctation of
aorta accounts for 5 to 8 % of patients with congenital
heart disease.[1] Marked increase in maternal cardiac output and oxygen
consumption in pregnancy is associated with left ventricular failure, aortic
dissection or rupture, endocarditis and cerebrovascular accidents. Individual
risk stratification depends upon the size of the aorta, presence of aneurysm,
severity of the coarctation and presence of hypertension.
Case
Report
A 23 year old primigravida with 38 weeks’ of
gestation was referred to our tertiary centre for intensive care in view of
breathlessness and fever with chills for 3 days. On examination, she had fever,
tachycardia (pulse of 136/minute), tachypnea (respiratory rate of 40/minute)
and hypertension - blood pressure (BP) of 150/80 mm Hg. On chest auscultation,
air entry was reduced in the left lower zone. She had been diagnosed as chronic
hypertension at 20 weeks of gestation and was on treatment with tablet
methyldopa 250 mg three times a day. She had scoliosis since birth.
All routine hematological
investigations were normal; however arterial blood gases revealed pH of 7.3
with oxygen saturation of 91%. Chest radiograph
showed patchy opacity in left lower zone suggestive of pneumonia. Chest
sonography showed minimal right sided pleural effusion with underlying
consolidation; hence a provisional diagnosis of community acquired pneumonia
was made. Cardiac cause of hypoxia was not suspected at this time. Obstetric
ultrasonography showed severe oligohydramnios.
The patient was started on intravenous
antibiotics and oxygen by mask as she was able to maintain her saturation only
with 100% oxygen. Antihypertensives were continued. On day 2 of admission, she
was transferred to intensive respiratory care unit in view of decreasing oxygen
saturation, where she went into spontaneous labor. At this time, in
view of differences in BP readings of both arms, right upper limb BP 150/90 mm
Hg and left upper limb BP 130/90 mm of Hg, emergency cardiology opinion
was taken and 2- D echocardiography was done, which diagnosed severe coarctation of aorta just proximal to the left
subclavian artery: diameter of aorta at coarctation site was 6 mm (compared to
14 mm at diaphragm), with peak systolic gradient of 100 mm of Hg across the
descending aorta and ejection fraction
of 50%. Infective endocarditis prophylaxis was started and emergency
LSCS was performed under epidural anesthesia, which
was difficult due to scoliosis. Male baby weighing 2.76 kg was delivered
with Apgar score of 9/10; liquor amnii was thick meconium stained. The patient
was shifted to intensive cardiac unit postoperatively, where her course was
uneventful. She was started on tablet metoprolol 12.5 mg daily, and discharged
after ten days. CT aortogram postpartum showed hypoplastic aortic arch, as seen
in figures 1 and 2. Aortoplasty is planned 6 months postpartum.
Figure 1: CT aortogram (AP and lateral) showing
coarctation in lateral view.
Figure 2: Magnified view of coarctation of aorta.
Discussion
The vascular malformation in coarctation is a
defect in the medial layer, giving rise to a prominent posterior infolding or
“posterior shelf”. Proliferation of elastic tissue in the intima occurs distal
to the coarctation causing intimal dissection or aneurysm formation.[2]
Coarctation may be associated with other
cardiovascular anomalies like bicuspid aortic valve, left-sided obstructive or hypoplastic defects and ventricular septal
defects, in almost 50% of patients.
Extracardiac nonvascular anomalies of head and neck, skeletal, genitourinary,
gastrointestinal, or respiratory systems may be present, as in our patient who
had thoracic spine scoliosis. Intracranial aneurysm of circle of Willis is seen in 3-5% cases.
Aortic coarctation
causes congestive heart failure in early life. In adults, it is usually
asymptomatic; however, hypertension, headache, nosebleed, leg cramps,
claudication, muscle weakness or neurologic changes may be seen. Clinical
examination is diagnostic: systemic hypertension is almost invariable with
differences in upper and lower extremity arterial pulses and BP; diminished and
delayed pulses distal to the obstruction are characteristic. Hence it should be
a routine practice in every patient
with systemic arterial hypertension to simultaneously palpate brachial and
femoral pulses to search for the “brachial-femoral delay” of significant aortic
coarctation. Supine bilateral arm (brachial artery) BP and prone right or left
supine leg (popliteal artery) BP should be measured to search for differential
pressures.
In pregnancy, increased
blood volume and increased estrogen levels contribute to hypertension and
increased shear stress on aortic wall; systemic hypertension is hence the most
common risk associated with patients of coarctation, and is responsible for
dilatation and dissection of the aorta. Poorly controlled hypertension also
leads to adverse neonatal outcomes like growth retardation, abruption and
premature delivery. Antihypertensive
therapy can cause hypotension distal to the coarctation site, leading to
reduction in uteroplacental perfusion.[3]
In unrepaired
coarctation, as also in repaired coarctation with arterial hypertension,
residual coarctation, or aortic aneurysm, the risk to the fetus and mother is
increased, and maternal mortality rate may be as high as 3-8%. Risk of aortic
dissection, rupture of cerebral aneurysm, congestive heart failure and angina
pectoris are increased in late pregnancy, intrapartum and within 6 weeks
postpartum due to hemodynamic and hormonal changes.
Conservative management is preferred
in antenatal period. Control of hypertension by beta-blockers, management of
congestive cardiac failure by digoxin and afterload reduction by diuretics
should be done as per individual case assessment. Indications
for surgical intervention include significant coarctation or re-coarctation
with long standing hypertension, hemodynamically significant aortic stenosis
and female patient contemplating pregnancy.
Percutaneous intervention is possible during
pregnancy. It
should be performed only if there is
persistence of severe resistant hypertension or presence of
maternal compromise;
as there is high chance of
dissection during procedure.[4] Surgical techniques
include balloon dilatation with or without stent placement, patch aortoplasty
with Dacron patch, and direct surgical repair with excision of the
para-coarctation tissue.
LSCS was previously
recommended due to greater risk of rupture of both aortic and cerebral artery
aneurysms in late pregnancy, labor and early puerperium following vaginal
delivery. However recent European Society of Cardiology
(ESC) guidelines suggest vaginal delivery under epidural anesthesia is safe in
both uncorrected and corrected coarctation.[4]
To conclude, coarctation was fortunately but
incidentally diagnosed when our patient was in labor. Value of measuring BP in
different limbs cannot be over emphasised in every patient of hypertension.
Contribution of physician, cardiologist and anesthetist was important in this
case, and possibility of aortic dissection and subsequent mortality was
averted.
References
1.
Yavuz C, Soydinc HE,
Tekbaş G and Karahan O. Pregnancy Complicated with Severe Recurrent Aortic
Coarctation: Case Rep Vasc Med 2012;
Article ID 865035. Available from: http://www.hindawi.com/journals/crivam/2012/865035/
2.
Wald RM, Sermer M, and
Colman JM. Pregnancy in young women with congenital heart disease:
Lesion-specific considerations. Paediatr Child Health. 2011;16:e33-e37.
3.
Connolly HM. Pregnancy
in women with coarctation of the thoracic aorta. ACC Curr J Rev 1997;55:6-7.
4.
Warnes CA, Williams
RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ESC Guidelines on the
management of cardiovascular diseases during pregnancy: 2011;32:3147–3197.
Citation
Dudhe M, Saxena N, Khadkikar R, Qureshi S, Chauhan AR. Near - Miss Mortality In Pregancy With Coarctation
Of Aorta. JPGO 2014 Volume 1 Number 8. Available from: http://www.jpgo.org/2014/08/near-miss-mortality-in-pregancy-with.html