Asymptomatic complete distal abdominal aortic occlusion with initial presentation of ruptured intracranial aneurysm.

Aortoilliac occlusive disease is occlusive atherosclerosis disease involving the distal aorta and bifurcation of iliac arteries and it is a subtype of peripheral arterial disease. Total occlusion of the abdominal aorta is a rare occurrence with an incidence of 3% -8.5% among the aortoiliac occlusive disease patients. We present a case of a 53 years old patient with a background history of hypertension and ex intravenous drug abuser with negative retroviral screening status, with no previous complaints who was brought to the Emergency Department with sudden onset of altered sensorium and 1 episode of seizure. Computed tomography angiogram of the brain showed a ruptured anterior communicating artery aneurysm. Diagnostic conventional angiogram of the brain was planned; however, difficulty was encountered during bilateral femoral artery cannulation with the abrupt termination of bilateral common iliac arteries. Computed tomography angiogram of the abdomen showed incidental finding of total occlusion of the abdominal aorta. As a conclusion, total occlusion of abdominal aorta secondary to aortoiliac occlusive disease with an associated intracranial aneurysm is never reported in the literature to date. This case highlights the possibility of association in between these two conditions which may benefit from further research.

Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

Aorto-left ventricular tunnel originating from the left coronary sinus with aortic aneurysm in an 11-year-old boy.

Aorto-left ventricular tunnel, characterized by extracardiac communication between the ascending aorta and left ventricle, is a very rare congenital condition. Although some affected infants remain asymptomatic until adulthood, most present with symptoms of heart failure during their first year of life. We report the case of an 11-year-old boy who had coexisting abnormalities: an aorto-left ventricular tunnel originating from the left coronary sinus, and an ascending aortic aneurysm. The patient underwent on-pump surgical correction and was in New York Heart Association functional class I status a year later. We also review the nature of this rare condition.

Aorta-right atrial tunnel.

Aorta-right atrial tunnel is a vascular channel that originates from one of the sinuses of Valsalva and terminates in either the superior vena cava or the right atrium. The tunnel is classified as anterior or posterior, depending upon its course in relation to the ascending aorta. An origin above the sinotubular ridge differentiates the tunnel from an aneurysm of the sinus of Valsalva, and the absence of myocardial branches differentiates it from a coronary-cameral fistula. Clinical presentation ranges from an asymptomatic precordial murmur to congestive heart failure. The embryologic background and pathogenesis of this lesion are attributable either to an aneurysmal dilation of the sinus nodal artery or to a congenital weakness of the aortic media. In either circumstance, progressive enlargement of the tunnel and ultimate rupture into the low-pressure right atrium could occur under the influence of the systemic pressure.The lesion is diagnosed by use of 2-dimensional echocardiography and cardiac catheterization. Computed tomographic angiography is an additional noninvasive diagnostic tool. The possibility of complications necessitates early therapy, even in asymptomatic patients or those with a hemodynamically insignificant shunt. Available treatments are catheter-based intervention, external ligation under controlled hypotension, or surgical closure with the patient under cardiopulmonary bypass.Herein, we discuss the cases of 2 patients who had this unusual anomaly. We highlight the outcome on follow-up imaging (patient 1) and the identification and safe reimplantation of the coronary artery (patient 2).

Ascending Aortic Rupture in a Young Woman with Loeys-Dietz Syndrome: The First Case Report in Korea / 대한흉부외과학회지

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-beta receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.

Congenital Anomalies of Aortic Arch: CT Angiography

Aortic arch anomalies result from the failure of an embryonic vascular structure to persist and regress in the usual manner during formation of the aortic arch. The anomalous aortic arch may encircle and compress the trachea and esophagus as a form of a vascular ring. The diagnosis of aortic arch anomaly and the recognition of airway compression are important because they are conditions which complicate the natural and surgical course of related diseases. CT can demonstrate the nature of anatomic structures such as the trachea and esophagus not revealed by angiography, simultaneously disclosing the relationship of stenotic airways and offending mediastinal vessels. Volumetric data acquisition by means of spiral CT enables three dimensional reconstruction,which can provide easy global understanding of the complex anatomy and spatial relationship of airway and cardiovascular structures. Three dimensional imaging is very useful for the physician and surgeon who are not accustomed to mentally reconstructing axial images, and can facilitate surgical planning.