Age: 41 years
Gender: Male
Lifestyle information: Aggressive sportsman
Working diagnosis: Pulmonary regurgitation
HISTORY
The patient underwent a successful surgical valvotomy for pulmonary valve stenosis at the age of seven.
He had a splenectomy following a motor-cross motorbike accident in his early 20s. He receives an annual pneumococcal vaccine and a small dose of penicillin prophylaxis daily.
He was well leading a full life until about 2 years ago, when he had his first episode of what was probably atrial fibrillation triggered by an upper respiratory tract infection. He was started on sotalol and converted spontaneously to sinus rhythm. Given the absence of arrhythmias on subsequent 24-hour ECG tape recordings, the medication was stopped.
He remained asymptomatic until 2 months ago, when he presented with chest pain and palpitations. He had a supraventricular tachycardia, and sotalol 80 mg twice daily was reintroduced.
Since then the patient has experienced palpitations clearly related to exercise and always resolving with rest. The symptoms did not improve after increasing the dose of sotalol. Eventually this was stopped and replaced with amiodarone 200 mg daily.
Comments: The patient was born with pulmonary valve stenosis. Surgical valvotomy was widely employed in the 1960s and 1970s. More recently, balloon valvuloplasty has become the treatment of choice for pulmonary valve stenosis. Surgical valvotomy is nowadays reserved for extremely dysplastic valves and patients with sub- or supravalvular stenosis.
Some degree of pulmonary regurgitation is found after surgical or balloon valvuloplasty of a stenotic pulmonary valve in up to 70% of cases, and is reported to be moderate or severe in 25% of them.
In these patients, as in patients with repaired tetralogy of Fallot, chronic severe pulmonary regurgitation may cause right ventricular dilatation and dysfunction, reduced exercise capacity, atrial and ventricular arrhythmias, and sudden cardiac death.
CURRENT SYMPTOMS
The patient complained of palpitations, most of which were clearly related to exertion and resolved with rest. Sometimes he would wake from sleep with a fast, irregular heart rate.
He also noted worsening of his exercise capacity. Although previously very fit, he was now becoming breathless after climbing one flight of stairs.
NYHA class: II
Comments: Pulmonary regurgitation is usually well tolerated, and patients remain symptom-free for many years. When patients become symptomatic, right ventricular systolic dysfunction is likely and may be irreversible.
CURRENT MEDICATIONS
Amiodarone 200 mg daily
Comments: The efficacy of amiodarone for maintaining sinus rhythm in patients with nonischemic heart disease appears to be better than that of sotalol ; amiodarone does, however, have more side effects, especially long term. Class I antiarrhythmic agents (such as flecainide and propafenone) may also be useful in preventing atrial fibrillation, but experience with this treatment is limited and some advocate against their use for patients with structural heart disease.
PHYSICAL EXAMINATION
BP 128/78 mm Hg, HR 85 bpm, oxygen saturation 99% on room air
Height 183 cm, weight 87 kg, BSA 2.1 m 2
Surgical scars: Sternotomy, laparotomy
Neck veins: Normal waveform, 2 cm above the sternal angle
Lungs/Chest: Clear
Heart: There was an RV lift. The heart rhythm was regular. There was a normal first heart sound and single second sound with no audible pulmonary component. A 2/6 systolic ejection murmur and a low-pitched early ending diastolic murmur were audible at the left sternal edge.
Abdomen: Normal
Extremities: Normal
Comments: An RV heave reflects RV dilatation. The absence of the pulmonary component of the second sound may reflect an absent, defective, or stenotic pulmonary valve. The systolic murmur reflects some degree of residual turbulence through the RVOT. The diastolic murmur of pulmonary regurgitation is best heard with the bell at the second or third left intercostal space. It is a low frequency murmur with a delayed onset after the second sound; its duration seems to be inversely related to the severity of pulmonary regurgitation. A short diastolic murmur, therefore, with an RV heave suggests severe pulmonary regurgitation and a dilated RV.
There were no signs of congestion in this patient. Elevated jugular pressure, hepatic enlargement, and peripheral edema can be present when there is established right ventricular dysfunction with decompensated heart failure.
LABORATORY DATA
| Hemoglobin | 14.9 g/dL (13.0–17.0) |
| Hematocrit/PCV | 43% (41–51) |
| MCV | 93 fL (83–99) |
| Platelet count | 285 × 10 9 /L (150–400) |
| Sodium | 140 mmol/L (134–145) |
| Potassium | 4.3 mmol/L (3.5–5.2) |
| Creatinine | 0.9 mg/dL (0.6–1.2) |
| Blood urea nitrogen | 6.1 mmol/L (2.5–6.5) |
Comments: No abnormalities were detected.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 84 bpm
QRS axis: +15°
QRS duration: 105 msec
Atrial flutter at a rate of 255 bpm and with 3 : 1 conduction. A degree of RBBB is present.
Comments: The patient was in atrial flutter with a normal ventricular rate at rest. Macroreentrant atrial tachycardias can often be atypical and subtle in ACHD, and the provider should be careful to inspect the ECG carefully.
It is speculative, but his AV conduction probably increases during exercise, accounting for his exertional palpitations. The atrial rate is slower than the usual atrial flutter rate of 300 bpm because the patient is on amiodarone.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 48%
Mild cardiomegaly, with normal pulmonary vascular markings. Straightening of the left heart border due to dilation of the RVOT and the main pulmonary artery. RA enlargement. Prior sternotomy.
Comments: RV enlargement and dilatation of the pulmonary trunk are the hallmarks of pulmonary regurgitation, though better appreciated on lateral projections (not available for this patient).
EXERCISE TESTING
| Exercise protocol: | Modified Bruce |
| Duration (min:sec): | 8 : 26 |
| Reason for stopping: | Tachycardia |
| ECG changes: | Tachycardia |
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