A 27-year-old man presents to the emergency department (ED) with a 3-day history of worsening epigastric pain and nonbilious vomiting. His bowel movements were normal until the day of presentation, when they turned black and sticky. He denies having any hematemesis, fever, chills, or any other associated symptoms, as well as any history of prior surgeries or medical problems. He has taken no medications recently except for Pepto-Bismol, which made the pain worse. He does not smoke tobacco but consumes 4 beers each day. He denies any illicit drug use.
On physical examination, his oral temperature is 98.6°F (37.0°C). His pulse has a regular rhythm with a rate of 88 bpm. His blood pressure is 198/88 mm Hg. He is noted to be in mild distress secondary to his epigastric discomfort. The examination of his head and neck, including a check for icteric sclerae, is normal. His lungs are clear to auscultation with normal respiratory effort. A 1/6 soft systolic ejection murmur is detected. His S1 and S2 heart sounds are normal. His abdomen is soft but tender to deep palpation in the epigastric region. The rectal exam reveals normal tone and black, guaiac-negative stool. The peripheral arterial pulses in the lower extremities are palpable but diminished when compared to the pulses in the upper extremities.
{mosimage}The laboratory analysis, including a complete blood count and a basic metabolic panel, is normal; however, his serum amylase and lipase levels are elevated, at 240 U/L (normal range, 30-110 U/L) and 2118 U/L (normal range, 46-218 U/L), respectively. The patient is diagnosed with alcohol-induced pancreatitis and treated in the ED with bowel rest, intravenous fluids, antiemetics, and generous doses of intravenous opiate analgesics. The patient is to be admitted to the hospital for continued bowel rest and intravenous fluid therapy for complete resolution of his pancreatitis; however, despite having his pain eased, the patient is noted to have a persistently elevated systolic blood pressure in the 190-199 range and a diastolic blood pressure in the 90-109 range. When further queried for his past medical history and a thorough review of systems, the patient does not recall ever having had his blood pressure checked, although he does report that he frequently experiences cramping in his legs and sometimes feels as if his feet are “cold.”
A posteroanterior chest radiograph is obtained (Image 1).
What is the most likely cause of this patient’s hypertension, and what further testing should be performed?
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HINT
The next step should be to check the blood pressure in the lower extremities.
Authors:
Girendra Hoskere, MD, Fellow, Division of Pulmonary Diseases and Critical Care Medicine, James H. Quillen College of Medicine, East Tennessee State University
Ryland P. Byrd, Jr, MD, Chief of Pulmonary Medicine, Medical Director of Respiratory Therapy, Quillen Mountain Home Veterans Affairs Medical Center, Professor, Department of Internal Medicine, Division of Pulmonary Diseases and Critical Care Medicine, James H. Quillen College of Medicine, East Tennessee State University
Thomas M. Roy, MD, Chief of the Division of Pulmonary Diseases and Critical Care Medicine and Professor of Medicine Department of Internal Medicine, James H. Quillen College of Medicine, East Tennessee State University
eMedicine Editors:
Brady Pregerson, MD, Dept. of Emergency Medicine, Cedars-Sinai Medical Center, Los Angeles, CA; Dept. of Emergency Medicine, Tri-City Medical Center, Oceanside, CA
Rick G. Kulkarni, MD, FACEP, Assistant Professor, Yale School of Medicine, Section of Emergency Medicine, Department of Surgery, Attending Physician, Medical Director, Department of Emergency Services, Yale-New Haven Hospital, CT
Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center, Seattle, Wash.
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ANSWER
Aortic coarctation: The patient’s hypertension and murmur are likely caused by a previously undiagnosed coarctation of the aorta. The chest radiograph demonstrates an enlarged collateral intercostal arterial circulation that has caused notching of the inferior-posterior rib margins, which is supportive of the diagnosis. Magnetic resonance angiography (MRA) confirms the presence of a postductal aortic coarctation (Image 2).
Coarctation of the aorta is a congenital condition that results in narrowing of a segment of the aorta. It was first described by Giovanni Morgagni in 1760. Historically, coarctation of the aorta was classified as preductal (before the origin of the ductus arteriosus) or juxtaductal (distal to the origin of the ductus arteriosus). More recently, the latter designation has been abandoned for the term postductal. The postductal form is the most common type of coarctation of the aorta. Coarctation of the aorta constitutes approximately 6-8% of all congenital heart diseases in infants. It is most frequently associated with other forms of congenital heart disease, such as ventricular septal defects, patent ductus arteriosus, bicuspid aortic valves, and aortic stenoses; however, it may also appear as an isolated condition. It is more common in males than in females; in patients with ovarian agenesis (Turner syndrome), it is particularly common.
Patients diagnosed with coarctation of the aorta in infancy usually have a combination of other heart anomalies that can lead to overt congestive heart failure. Those patients presenting beyond infancy, such as in this case, often have vague symptoms that may include headaches, a propensity to nose bleeds, leg cramps, and cold feet. Hypertension is usually present and leads to further in-depth testing. The hallmark physical sign of postductal coarctation is that blood pressure in the arms is at least 20 mm Hg higher than it is in the lower extremities (normally blood pressure in the lower extremities is slightly higher than in the upper extremities). A systolic or continuous murmur in the infrascapular or infraclavicular area also may suggest the presence of this anomaly. Additional murmurs may indicate the presence of associated anomalies, such as ventricular septal defects, patent ductus arteriosus, or aortic stenoses.
Chest radiography may demonstrate cardiomegaly and rib notching from the compensatory collateral intercostal arterial dilatation. The classic “figure 3” sign seen on chest radiographs occurs in at least one third of patients, and results from prestenotic dilatation of the ascending aorta followed by indentation of the aorta at the coarctation site and poststenotic dilatation of the descending aorta. The “reverse 3” or “E” sign can also be observed on barium swallow studies, resulting from matched compression of the esophagus by the dilated segments of the aorta. Signs of left ventricular hypertrophy are also often seen on electrocardiograms (ECGs). An echocardiogram is usually performed to detect any associated cardiac anomalies. MRA and cardiac catheterization may be necessary to confirm the exact location and the presence of collaterals. A gradient of more than 20% across the stenosis during cardiac catheterization indicates a severe coarctation and requires urgent intervention.
Treatment of aortic coarctation is usually surgical. Antihypertensive medication must be used with caution, as its use may lead to inadequate perfusion of the lower body and renal impairment. The best treatment strategy is admission for early repair. If left untreated, 90% of patients with aortic coarctation die by the age of 50 years, usually from hypertensive complications. Historically, surgical resection of the involved aortic segment has been the treatment of choice, and it has a high success rate. More recently, balloon dilatation and placement of endovascular stents are gaining popularity and becoming accepted forms of primary therapy, especially for patients who may be unsuitable candidates for surgery. Postoperative complications may be acute or delayed and include persistent hypertension, recoarctation, and aortic aneurysms, among others.
This patient had an echocardiogram that failed to reveal any associated cardiac defects. He was referred for surgical intervention and underwent successful resection of the coarcted aortic segment after resolution of his associated pancreatitis, the initial reason for his presentation and admission.
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