Precordial catch syndrome

Precordial catch syndrome refers to a common cause of pediatric chest pain that was first described in 1955 by Miller and Texidor. It is often referred to as Texidor twinge. The pain is described as a sudden onset, sharp, stabbing, midsternal or precordial chest pain without radiation. Patients generally can point to the area of maximal pain, although the pain or tenderness is often absent at the time of evaluation, because the pain characteristically resolves without intervention. Typical duration of the pain is from 30 seconds to 3 minutes. The pain may be exacerbated by deep breathing, but it is not associated with dyspnea, cough, or other respiratory findings. The most common age of presentation for precordial catch syndrome is 6 to 12 years.

The pathophysiology of precordial catch syndrome is unknown, although it is hypothesized to originate from the parietal pleura, from rib cartilage, or the chest musculature and boney structures. Like most causes of chest pain, there is a broad differential when encountered in the pediatric patient. Actual diagnosis is made with a thorough history, lack of findings on physical examination, and normal ancillary testing, although electrocardiography and chest radiography are not indicated as part of the initial evaluation of precordial catch syndrome. If history and physical examination alone cannot rule out other causes of chest pain, such as spontaneous pneumothorax, rib fractures, or pericarditis, then ancillary tests may be necessary.

Management and treatment of patients diagnosed with precordial catch syndrome is supportive. Family and patient anxiety may be high surrounding chest pain, particularly if there is a family history of cardiovascular disease. Reassurance is required. Analgesic medications may not be helpful, as the pain is self-limiting and of short duration.

Chest pain associated with cocaine or methamphetamine use

Illicit drug use is frequent, with an estimated 19.9 million illicit drug users in 2007, which represented 8% of the population over 12 years of age. After marijuana, cocaine was the second most commonly used drug in the United States, accounting for 0.8% of the population over the age of 12, which equates to 2.1 million users. Overall, rates of illicit drug use among 12- to 17-year-olds have decreased in the United States, from 11.6% in 2001 to 9.5% of the population in 2007. A similar decrease was seen in 18- to 25-year-olds, although the use of prescription pain medications has recently been on the rise.

Chest pain associated with both cocaine and methamphetamine use is described in the literature, and it is important for health care workers to remember that people often combine illicit drugs and concomitantly use tobacco, alcohol, or prescription medications. Chest pain associated with cocaine use is often described as tight rather than sharp, is typically confined to the chest and arm, and can be associated with diaphoresis, nausea, vomiting, or dyspnea. The incidence of these symptoms is similar to patients presenting with cardiac chest pain.

The incidence of acute myocardial infarct with cocaine use is as high as 6% in adults. However, the incidence with the use of methamphetamine and other illicit drugs is not reliably reported in the literature. Pediatric incidence of acute myocardial infarction due to cocaine use is unknown, although screening for illicit drugs in teenagers with chest pain should be considered. The pathophysiology of chest pain associated with illicit drug use is commonly coronary artery vasospasm, which may or may not be associated with underlying atherosclerotic or congenital cardiac disease, having implications for treatment options. Cocaine acts as a sympathomimetic agent by blocking the reuptake of norepinephrine and dopamine at presynaptic adrenergic terminals, leading to excessive postsynaptic stimulation, thus increasing overall oxygen demand by the heart. Cocaine also leads to premature coronary thrombus formation and is associated with early onset coronary artery disease and premature coronary artery plaque formation.

Adult and pediatric patients are unreliable at self-reporting drug use. Physiologic changes seen on physical examination include elevated systolic and diastolic blood pressures with increased mean arterial pressures compared with noncocaine users presenting with chest pain. Heart rate is increased due to sympathomimetic effects, and patients may exhibit tachypnea and diaphoresis. Electrocardiographic findings consistent with acute coronary ischemia, arrhythmias, or pericarditis may be found, and serum cardiac enzymes may be elevated in patients with chest pain who use cocaine or methamphetamine. Angiography frequently is normal, with no or minimal evidence of atherosclerotic cardiac artery disease.

Treatment of chest pain associated with ischemia induced by cocaine is similar to patients presenting with acute coronary syndrome, and should include antiplatelet drugs such as aspirin and clopidogrel. Beta-blockers should be avoided, as unopposed alpha-adrenergic stimulation can lead to increased coronary artery vasospasm and worsening cardiac circulation and ischemia. Benzodiazepines should be instituted as an early management strategy, because they decrease the central stimulation of cocaine, decrease actual chest pain, and can improve cardiovascular hemodynamics. Nitroglycerine also has been shown to decrease coronary artery vasospasm in patients who have used cocaine and present with evidence of ischemia. Angiography frequently is normal, with no or minimal evidence of atherosclerotic cardiac artery disease. Long-term, ongoing drug treatment may be required for these patients.

Chest pain associated with cocaine or methamphetamine use

Illicit drug use is frequent, with an estimated 19.9 million illicit drug users in 2007, which represented 8% of the population over 12 years of age. After marijuana, cocaine was the second most commonly used drug in the United States, accounting for 0.8% of the population over the age of 12, which equates to 2.1 million users. Overall, rates of illicit drug use among 12- to 17-year-olds have decreased in the United States, from 11.6% in 2001 to 9.5% of the population in 2007. A similar decrease was seen in 18- to 25-year-olds, although the use of prescription pain medications has recently been on the rise.

Chest pain associated with both cocaine and methamphetamine use is described in the literature, and it is important for health care workers to remember that people often combine illicit drugs and concomitantly use tobacco, alcohol, or prescription medications. Chest pain associated with cocaine use is often described as tight rather than sharp, is typically confined to the chest and arm, and can be associated with diaphoresis, nausea, vomiting, or dyspnea. The incidence of these symptoms is similar to patients presenting with cardiac chest pain.

The incidence of acute myocardial infarct with cocaine use is as high as 6% in adults. However, the incidence with the use of methamphetamine and other illicit drugs is not reliably reported in the literature. Pediatric incidence of acute myocardial infarction due to cocaine use is unknown, although screening for illicit drugs in teenagers with chest pain should be considered. The pathophysiology of chest pain associated with illicit drug use is commonly coronary artery vasospasm, which may or may not be associated with underlying atherosclerotic or congenital cardiac disease, having implications for treatment options. Cocaine acts as a sympathomimetic agent by blocking the reuptake of norepinephrine and dopamine at presynaptic adrenergic terminals, leading to excessive postsynaptic stimulation, thus increasing overall oxygen demand by the heart. Cocaine also leads to premature coronary thrombus formation and is associated with early onset coronary artery disease and premature coronary artery plaque formation.

Adult and pediatric patients are unreliable at self-reporting drug use. Physiologic changes seen on physical examination include elevated systolic and diastolic blood pressures with increased mean arterial pressures compared with noncocaine users presenting with chest pain. Heart rate is increased due to sympathomimetic effects, and patients may exhibit tachypnea and diaphoresis. Electrocardiographic findings consistent with acute coronary ischemia, arrhythmias, or pericarditis may be found, and serum cardiac enzymes may be elevated in patients with chest pain who use cocaine or methamphetamine. Angiography frequently is normal, with no or minimal evidence of atherosclerotic cardiac artery disease.

Treatment of chest pain associated with ischemia induced by cocaine is similar to patients presenting with acute coronary syndrome, and should include antiplatelet drugs such as aspirin and clopidogrel. Beta-blockers should be avoided, as unopposed alpha-adrenergic stimulation can lead to increased coronary artery vasospasm and worsening cardiac circulation and ischemia. Benzodiazepines should be instituted as an early management strategy, because they decrease the central stimulation of cocaine, decrease actual chest pain, and can improve cardiovascular hemodynamics. Nitroglycerine also has been shown to decrease coronary artery vasospasm in patients who have used cocaine and present with evidence of ischemia. Angiography frequently is normal, with no or minimal evidence of atherosclerotic cardiac artery disease. Long-term, ongoing drug treatment may be required for these patients.

Aortic dissection

Aortic dissection is rare in the pediatric population, with estimates being less than 0.1% of chest pain cases. Aortic dissection, however, can be life threatening if not recognized early and treated aggressively. Aortic dissection can be associated with various pediatric conditions, including trauma, Marfan syndrome, Ehlers-Danloss syndrome, congenital bicuspid aortic valve, coarctation of the aorta, vasculitis, cocaine use, recent cardiac surgery, or aortic cannulation as seen with congenital heart disease. Traumatic aortic dissection accounts for nearly 50% of cases of aortic dissection under the age of 21 years, and Marfan syndrome accounts for roughly 25% of cases. In Marfan syndrome, aortic dissection is associated with aortic root dilatation, and the incidence is increased during pregnancy. Cocaine and other illicit drug use likely contribute to the increased incidence of aortic dissection through various mechanisms, most commonly unchecked hypertension.

An aortic root diameter of greater than 6 cm in adults is associated with the greatest risk of rupture, and intervention is recommended for aortic root diameters of greater than 5.5 cm. In trauma, however, there is usually no dilatation, but rather the force of the impact may disrupt the aortic root.

The pain typically is described as a sudden onset, sharp, “tearing” or “ripping” pain that may be anterior in the chest or radiating to the back. Patients also may present with abdominal pain, depending on the extent of the dissection, and more rarely with syncope or extremity and organ blood flow disruption from the extent of the dissection.

In someone with a recent history of surgery, pneumonia, pulmonary embolism, or complications of surgery should be part of the differential for the cause of chest pain. For a patient with Marfan syndrome, it is important to also consider spontaneous pneumothorax as a cause of chest pain. Patients with congenital heart disease additionally may have disease-specific considerations, pericardial effusions, or infectious etiologies for chest pain.

The diagnostic gold standard for aortic dissection had been aortic angiography; however, in recent years computed tomography (CT), cardiovascular magnetic resonance imaging, and cardiac ultrasound all have been shown to be accurate and less invasive methods for the diagnosis of aortic dissection. Chest radiography may show a widening of mediastinal structures in up to 85% of patients for those with traumatic aortic disruptions or high thoracic aortic dissections. Electrocardiographic findings may suggest left ventricular strain, hypertrophy, cardiac ischemia, nonspecific ST-T wave changes, or even an acute myocardial infarct pattern.

Initial treatment involves controlling both blood pressure and heart rate to minimize the stress on the wall of the aorta while preparing for definitive operative repair. The goal values for adult systolic blood pressure are between 100 and 120 mm Hg, with a heart rate around 60 beats per minute. The goal values for vital signs should be appropriately age-adjusted in the pediatric population. Beta-blockers are the mainstay of therapy, as they also control blood pressure and heart rate, although peripheral vasodilators also can be helpful in decreasing blood pressure and left ventricular contractility forces. Many beta-blockers, such as labetalol, have extremely short half-lives. Sodium nitroprusside is commonly used in pediatric hypertensive crises, but should not be used as monotherapy in acute aortic dissections, because it can raise the peak maximal rate of pressure rise in the left ventricle during contraction, thus potentially expanding or rupturing an aortic dissection.

For patients with underlying medical conditions such as Marfan syndrome, primary prevention with control of hypertension, aortic root dilatation monitoring, and early intervention is the best option. Definitive treatment of acute aneurysms requires prompt recognition, blood pressure and pulse control, and definitive surgical intervention. Despite monitoring and prompt evaluation, acute aortic dissections have a very high mortality rate.

Chest tumors/malignancy

Primary tumors arising from mediastinal structures are rare, and more commonly occur in the adult population. The average age of presentation for patients with mediastinal tumors is 30 years old. The ratio of males to females is roughly 2.8:1. Chest tumors in the pediatric population are likely to be neuroblastoma, lymphoma, or primary neurectodermal tumors (PNET). The location of the tumor (anterior, middle, or posterior) within the mediastinum can suggest possible etiologies. Lymphoma, teratomas, thymomas, and thyroid tumors are more likely in the anterior and middle mediastinum, and neuroblastoma, neurofibroma, sarcoma, and germ cell tumors are more common in the posterior mediastinum.

Patients may present with cough, wheezing, stridor, and anterior chest pain that is characterized by a deep sensation. Chest pain is found in half of all patients who present with a new diagnosis of mediastinal tumor, and it is usually not associated with activity or movement. Respiratory symptoms may be related to activity, and cough is found in over 80% of patients. Less commonly, supraclavicular lymphadenopathy, pleural effusions, symptoms consistent with superior vena cava syndrome, or respiratory distress or failure may be the presenting symptoms.

Identification of the mass may be made by chest radiography or CT, but a biopsy is needed to confirm mass type, malignancy status, and treatment options. Fine needle aspiration, percutaneous lymph node biopsy, serum tumor markers, and mediastinoscopy or thoracostomy are all methods of obtaining tissue samples when chest masses have been identified. Individual therapy and outcome depend on the severity of the presentation and etiology of the tumor.

Chest pain with leukemia has been reported and may be due to mediastinal mass, mediastinal lymphadenopathy, focal bony destruction, pathologic fracture, pericarditis, pulmonary infarction from leukemic sludging, or coronary ischemia from direct infiltration of leukemic cells into the coronary arteries. In one case report, a 7-year-old with leukemia presented with vague, reproducible chest pain initially thought to be caused by costochondritis. He had persistent pain, but a rather unremarkable examination, and it is not clear what prompted the clinicians to obtain a complete blood count, which revealed the diagnosis.