Introduction:
Interrupted aortic arch (IAA) is a rare congenital aortic anomaly due to a complete interruption of aortic lumen between the ascending and descending aorta 1. It has a high mortality in infants accounting for 1% of congenital cardiovascular diseases. Approximately 75% of patients with IAA died by 10 days after birth and 90% died within the first year after birth 2. Based on the presence or absence of other associated deformities, IAA can be categorized as complex and isolated types. The former is more common and usually associated with ventricular septal defect and patent ductus arteriosus 3,4.The latter is rare, and patients with isolated IAA rarely survive to adulthood without surgical intervention unless the extensive joining of the collateral vessels to the descending aorta 5.Patients receiving operations in infancy were in diminished health status with significant diminished CPET performance and preserved resting LV function.The operative re-intervention rates was 29% and 18% required LVOT interventions over 15 years of follow-up 6.In this article, we will present three cases of adults with isolated interrupted aortic arch, and discuss about the theupatic strategy that whether surgical intervention is the best option for adult patients.
Materials and Methods:
Case presentation: Hospital database was searched for adult patients who
had a discharge diagnosis of IAA at Wuhan Union Hospital over the past
10 years based on the international classification of diseases codes.
The clinical notes of each potential case were reviewed, and patients
younger than 14 years old or with other cardiac malformations were
excluded. We also conducted a comprehensive literature search regarding
IAA. Wuhan Union Hospital institutional review board approved this
study.
3 patients were identified who were diagnosed with IAA at our
institution. They were admitted to our department with the chief
complaint of hypertension and later diagnosed with IAA using computated
tomography angiography(CTA) or transcatheter angiography. Besides
hypertension, they have no other symptoms. Two patients declined
surgical treatment and received conservative therapy including
anti-hypertensive treatment. The third patient received an
extra-anatomic bypass surgery from ascending aorta to descending aorta.
His post-surgical blood pressure remained high and was treated with
irbesartan and hydrochlorothiazide.
Case 1:A 43-year-old man was admitted to our department with the
complaint of high blood pressure in 2020. Physical examination showed
regular heart beat with blood pressure of 179/102 mmHg in the right arm,
170/100 mmHg in the left arm, 135/80 mmHg in the right limb and 125/70
mmHg in the left limb, and there was a systolic murmur around the
sternum. Electrocardiogram showed sinus rhythm. Echocardiography showed
possible coarctation of aorta without other cardiac malformations. Then
he was referred to the radiology department to further evaluate a
possible aortic abnormality. CTA showed an interruption of the aortic
arch distal to the origin of the left subclavian artery(Type A, Fig1 A
and B) and profuse collateral circulation. The blood biochemistry
laboratory results showed normal renal and liver function. There were no
signs or symptoms of ischemia of splanchnic organs. Since the patient
had no symptoms other than hypertension, we recommended
anti-hypertensive management. After taking valsartan amlodipine and
felodipine, his daily blood pressure in the left arm was stabilized at
about 140/90 mmHg.
Case 2:Another 43-year-old man was admitted to our department for
further treatment after he was diagnosed with IAA in another hospital in
2017. His physical examination showed regular heart beat with blood
pressure of 132/96 mmHg in the right arm, 130/92 mmHg in the left arm,
110/80 mmHg in the right limb and 108/70 mmHg in the left limb, and
there was a systolic murmur around the sternum. Before his referral, he
had had high blood pressure for one year with the highest systolic
pressure in the left arm up to 180 mmHg. Before he came to our
department, he had already been diagnosed with IAA by another hospital
and had been taking anti-hypertensive drugs for several weeks. His
electrocardiogram showed in sinus rhythm with incomplete right bundle
branch block. His echocardiography indicated possible coarctation of
aorta with widened STJ (sinotubular junction). CTA revealed a
coarctation or interruption of descending part of aortic arch with
ascending aortic aneurysm and abundant collateral circulation. To
determine whether it was a coarctation or an interruption, we performed
an aortic angiography via right femoral artery and right radial artery,
confirming that it was an interruption of the aortic arch distal to the
origin of the left subclavian artery (Type A) with profuse collateral
circulation(Fig1 C and D). His blood biochemistry laboratory results
showed normal renal and liver function, without signs of ischemia of
splanchnic organs. Similar to case 1, we recommended anti-hypertensives
with perindopril, and the patient’s daily blood pressure in the left arm
has stabilized at about 130/80 mmHg.
Case 3:A 28-year-old man was admitted to our department with the
complaint of high blood pressure in 2017. Physical examination showed
irregular heart beat with blood pressure of 174/110 mmHg in the right
arm, 180/108 mmHg in the left arm, 130/82 mmHg in the right limb and
135/84 mmHg in the left limb, and there was a systolic murmur around the
sternum. Electrocardiogram showed in sinus arrhythmia. Before he came to
our hospital he had had echocardiography and CTA. His echocardiography
showed coarctation of the descending aorta without other cardiac
malformations, and CTA showed coarctation of the descending aorta with
abundant collateral circulation. Initially, we attempted to place a
stent via aortography. However, the aortography via right femoral artery
and right radial artery showed an interruption of the aortic arch distal
to the origin of the left subclavian artery (Type A) with abundant
collateral circulation(Fig1 E and F ). The blood biochemistry results
showed normal renal and liver function without signs of ischemia of
splanchnic organs. Since the patient had no symptoms other than high
blood pressure, as case 1 and 2, we recommended anti-hypertensives.
However,given this patient’s young age and concerning regarding possible
future IAA-related complications such as heart failure, renal and liver
damage, etc., the patient and his family requested a surgery to correct
the aortic deformities. We performed a surgical reconstruction to
restore the continuity of aortic arch using extra-anatomic bypass from
ascending aorta to descending aorta with 16mm artificial blood vessel.
The surgery went well, but the patient’s systolic pressure in the right
arm remained at approximately 170 mmHg. He received anti-hypertensives
and accomplished satisfactory blood pressure (about 140/90 mmHg in the
left arm), which remained adequate, 2 years after the surgery. And the
patient was comatose for 2 weeks after the surgery. Since then he had
been with right limb weakness and suffered from epilepsy. Significant
diminished CPET performance and decreased communication ability
accompanied. He could only walk slowly for about 50 meters and have a
simple chat with others.