A 36-year-old female with known Eisenmenger syndrome (ES) is admitted to the ICU after 2-day hospital course for worsening hypoxia. She is followed as an outpatient in the cardiology clinic, and it is noted that her oxygen levels are usually low. Her vital signs show a heart rate of 95 bpm, oxygen saturation of 86% on noninvasive positive pressure ventilation, blood pressure of 116/80 mm Hg, and a respiratory rate of 18. On examination, you note the female in mild respiratory distress with signs of cyanosis. Chest X-ray showed mild pleural effusions bilaterally but no concern for pulmonary edema. Transthoracic echocardiography done the prior day showed concern for biventricular dysfunction.
What is the next best step to confirm this patient’s diagnosis?
Correct Answer: B
This patient is exhibiting signs of acute respiratory distress leading to respiratory failure with a known history of ES. Given this history, she likely is suffering from pulmonary arterial thrombosis and requires a CTA of her chest to confirm the diagnosis. ES is a consequence of severe PAH, more commonly as a result of congenital heart disease. It is most commonly associated with atrial and/or ventricular septal defects. The primary pathophysiology results in left to right shunting. This leads to shearing forces and stress on the pulmonary vasculature, resulting in endothelial dysfunction, release of inflammatory mediators, and vascular remodeling. The increase in pulmonary vascular resistance causes a right-to-left shunt that leads to cyanosis, which completes the picture of ES. TEE will only show the shunting and might not reveal the thrombosis (A). Cardiac catheterization will reveal the elevated pulmonary pressures but will not be diagnostic for a pulmonary venous thrombosis (C). Because the patient has only mild pleural effusions, a chest radiograph will not be very useful either (D).
Patients with ES are at risk for multiple comorbidities, including pulmonary arterial thrombosis (∼20%). Risk factors that further increase this risk include biventricular dysfunction, female sex, dilation of the pulmonary arteries, and decreased pulmonary blood flow.
You are posted in the Neonatal intensive care unit of the hospital. You get an emergency page that a pregnant patient has just delivered in the Emergency Department, and the child has a congenital cardiac condition for which he will need a Fontan procedure. The Fontan surgery, which involves the anastomosis of the right atrium to the pulmonary artery, is a useful surgical treatment for each of the following congenital cardiac defects EXCEPT:
Correct Answer: D
Truncus arteriosus occurs when there is presence of a single arterial trunk, overriding both ventricles (which are connected via a ventricular septal defect). This trunk gives rise to both the aorta and pulmonary artery. Surgical treatment of this defect includes banding of the left and right pulmonary arteries and enclosure of the associated VSD, and hence the Fontan would not be employed in this situation.
The Fontan procedure (usually modified Fontan) refers to the anastomosis of the right atrial appendage (or the superior and inferior vena cava) to the pulmonary artery. Because this procedure leads to an increase in pulmonary blood flow, it as the treatment of choice for treatment of congenital cardiac defects, which decrease the pulmonary artery blood flow (eg, pulmonary atresia and stenosis [C and E] and tricuspid atresia[A]). The Fontan procedure is also used to divert systemic venous return to pulmonary artery when it is necessary to surgically convert the right ventricle to a systemic ventricle (eg, hypoplastic left heart syndrome[B]).
A 15-year-old male patient was admitted to the ICU with a diagnosis of stroke after he got some accidental air bubbles injection while receiving IV fluid resuscitation in the emergency department, where he was seen for a viral prodrome. The patient’s family mentions that he has a known cardiac condition from birth, even though they do not know the name. Accidental injection of air into a peripheral vein would be LEAST likely to result in arterial air embolism in the patient with which of the following anatomic cardiac defects?
Correct Answer: A
Patent ductus arteriosus (A) causes left to right shunting and, hence, has the least risk of causing arterial air embolism. The anesthetic management of patients with congenital heart disease requires thorough knowledge of the pathophysiology of the cardiac defect. They can be categorized into defects causing left to right intracardiac shunts (acyanotic) and right to left shunts (cyanotic). The common congenital heart defects that result in right to left shunting include tetralogy of Fallot(C), Ebstein malformation of the tricuspid valve, pulmonary atresia with a ventricular septal defect (D), ES (B), tricuspid atresia (E), and patent foramen ovale. Meticulous care must be taken to avoid infusion of air via intravenous solutions because this can lead to arterial air embolism. Patients with congenital defects that result in left to right intracardiac shunting, such as patent ductus arteriosus, are at minimal risk for arterial air embolism because blood flow through the shunt is primarily from the systemic to the pulmonary system.
A patient with known pulmonary arterial hypertension (PAH) from congenital heart disease has recently been diagnosed with ES.
What is the most appropriate treatment that should be initiated in this patient?
Patients with congenital heart disease are at increased risk and incidence of development of PAH. The mortality remains high among these patients if left untreated. More recently, safe and effective drug therapy (disease targeted therapy) has become available for the treatment of PAH, which includes endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids. Disease-targeted therapies such as sildenafil and bosentan have been associated with improved survival at 10 years as shown in the BREATH-5 Trial. It has been shown that disease-targeted therapies for PAH results in better survival when comparted to traditionally used drugs such as digoxin, beta blockers, and calcium channel blockers. And disease-targeted therapies are the mainstay of treating PAH. Patients with PAH due to ES has a better survival than patient’s with primary PAH. This may be due to the fact that right ventricular function in patients with ES, and resultant PAH is better than those with idiopathic PAH.
A 23-year-old male presents to the emergency department after collapsing during a pick-up basketball game. On examination you find a young male in no apparent distress. He exhibits a harsh, systolic crescendo-decrescendo murmur. His EKG is an unremarkable save for short, sharp Q-waves in the lateral leads.
Which of the following is most strongly associated with this diagnosis?
This patient’s history strongly suggests hypertrophic obstructive cardiomyopathy (HOCM). Of the listed options, family history has the strongest risk association with sudden death from HOCM. HOCM is an autosomal dominant genetic disorder that affects the beta-myosin chains leading to asymmetric septal wall hypertrophy. This leads to a leftventricular outflow obstruction and the subsequent symptoms. Symptoms are typically effort related, including exertional angina, dyspnea, syncope, and sudden death. Heart failure can develop over time because of stiffening of the left ventricle and development of left-ventricular hypertrophy. EKG findings include LVH as well as a “pseudo infarction pattern” evidenced by “dagger-like” Q waves in the lateral or inferior leads. These pathognomonic findings are not associated with smoking, drugs, or alcohol use (B, C, and D). Treatment includes beta-blockers or calcium channel blockers, surgical myectomy, or cardiac ablation of portions of the septum.
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