44. Answer: E
All of the signs suggest pulmonary hypertension, pointing to Eisenmenger's syndrome, which may be found in older adults who have had a reversal of shunt from right‐to‐left before open‐heart surgery is available. It is the most advanced form of pulmonary arterial hypertension due to elevated pulmonary vascular resistance causing right‐to‐left intracardiac shunt or great artery shunting, leading to systemic arterial desaturation.
45. Answer: G
Tetralogy of Fallot is associated with a combination of four clinical features:
1 VSD
2 Right ventricular outflow obstruction
3 Overriding aorta
4 Right ventricular hypertrophy
Although the long‐term survival has improved for patients with repaired tetralogy of Fallot, residual haemodynamic and electrophysiological sequalae are common in adults. These groups of patients may have symptoms of arrythmias, heart failure, exercise intolerance, and death in early adulthood. Implantable cardioverter–defibrillators (ICDs) as a primary intervention should be considered in patients who meet standard qualifying criteria (i.e. LV ejection fraction ≤35% with NYHA class II or III symptoms).
46. Answer: D
The most common site for coarctation (CoA) is just distal to the origin of the left subclavian artery. An ECG may show signs of systolic overload, including left ventricular hypertrophy.
Sometimes the diagnosis can be easily missed if the lower limb blood pressure is not routinely measured. The long‐term complications of CoA are generally related to chronic upper body systemic hypertension. Complications in patients with repaired CoA include recoarctation of the aorta, aneurysm, pseudoaneurysm, and dissection; thus, patients will require ongoing monitoring after operation. CoA is also known to be associated with Turner's syndrome.
47. Answer: A
There are two common types of ASD in adults: ostium secundum (most common) and ostium primum. These patients characteristically exhibit a left‐to‐right intracardiac shunt which may lead to right heart enlargement and, in a minority of the patients, pulmonary arterial hypertension (PAH). ECG findings may demonstrate right‐axis deviation, right bundle branch block pattern, right ventricular hypertrophy from systolic overload and CXR findings may show an enlarged right atrium and ventricle, increased pulmonary vasculature, a dilated main pulmonary artery, and a small aortic knob. Almost all ASDs need to be closed surgically or, if the patient is suitable, with a percutaneous closure device when a pulmonary‐to‐systemic blood flow (shunt) ratio (Qp/Qs) >1.5:1. It is important to evaluate severe PAH prior to operation since closure of ASDs is contraindicated in this group of patients.
ASD due to ostium primum is caused by an endocardial cushion defect adjacent to the atrioventricular valves. In addition to the aforementioned signs in patients with ostium secundum, there is also associated mitral regurgitation, tricuspid regurgitation or VSD. ECG findings should show left‐axis deviation, right bundle branch block and sometimes a prolonged PR interval. The condition is associated with Down's syndrome and Holt‐Oram syndrome.
48. Answer: B
VSD in adults are usually small or large with Eisermenger's syndrome. Small restrictive defects may be monitored conservatively without the need for operation. ECG and CXR findings may demonstrate left ventricular hypertrophy. Closure of the VSD is indicated when the left‐to‐right shunt is moderate to large when the pulmonary‐to‐systemic flow is >1.5 to 1. Eisermenger's syndrome and severe PAH are contraindications to operative intervention due to significantly increased surgical risks. VSD is associated with Down syndrome.
49. Answer: F
A Marfanoid habitus is suggestive of a diagnosis of Marfan's syndrome. Patients with Marfan's syndrome are at an increased risk of experiencing progressive aortic root dilatation and aortic dissection. Serial echocardiograms are required to monitor the size of the aortic root over time. A slit‐lamp examination may be required to diagnose lens dislocation. Patients with lens dislocation may have had lens replacement surgery in the past.
50. Answer: C
The ductus arteriosus usually closes shortly after birth. Failure of the ductus to close in the early weeks of life results in a PDA, which allows blood to flow between the aorta and the pulmonary artery, increasing blood flow in the pulmonary circulation. Patients with a large PDA can exhibit signs and symptoms of pulmonary hypertension and heart failure. Patients with a small PDA are often asymptomatic but may develop infective endocarditis. PDAs are most commonly closed using catheter‐based or surgical intervention. An enlarged left ventricle and calcification of the duct may be evidenced on CXR.
Stout K, Daniels C, Aboulhosn J, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14).
https://ahajournals.org/doi/pdf/10.1161/CIR.0000000000000603
2 Critical Care Medicine
Questions
Answers can be found in the Critical Care Medicine Answers section at the end of this chapter.
1 1. Which of the following treatment options reduces the mortality of acute respiratory distress syndrome (ARDS)?Aggressive intravenous fluid administration for reversal of shock.Intravenous albumin administration.Intravenous hydrocortisone administration.Lung‐protective invasive mechanical ventilation with lower tidal volumes and airway pressures.
2 2. A 70‐year‐old man returns to the cardiology ward from the operating theatre after a pacemaker implantation for complete heart block. He complains of shortness of breath and chest pain. On examination, his HR is 120/min, BP is 90/70 mmHg and his respiratory rate is 30/min. His JVP is markedly elevated and his heart sounds are quiet. Air entry is symmetrical on lung auscultation. Oxygen saturation is 90% on room air. An ECG shows sinus tachycardia but is otherwise within normal limits.What is the next most appropriate investigation?CXR.CT of the chest.Echocardiogram.Serial troponin levels.
3 3. Which of the following clinical findings would be UNUSUAL in severe acute carbon monoxide poisoning?Chest pain.Headache.Loss of consciousness.Low oxygen saturation on pulse oximetry.
4 4. A 42‐year‐old man is admitted to the intensive care unit with severe sepsis. Regarding the diagnosis of disseminated intravascular coagulation (DIC), which of the following statements is most correct?Abnormal prothrombin time is required to diagnose DIC.A low fibrinogen level (<100 g/mL) is present in about 30% of septic patients with DIC.DIC affects less than 20% of patients with septic shock.The most frequent clinical manifestation of DIC in sepsis is haemorrhage.
5 5. Which type of circulatory shock is the most common in patients in the intensive care unit?Cardiogenic shock.Distributive shock.Hypovolaemic shock.Obstructive
