Age: 57 years
Gender: Male
Occupation: Logistics planner
Working diagnosis: Pulmonary stenosis with prior Ross repair
HISTORY
Aortic regurgitation was diagnosed when the patient was 23 years old. The patient underwent a Ross procedure 6 months later. He recovered uneventfully.
Ten years postoperatively he developed symptoms of rapid palpitation. No arrhythmias were documented. Coronary angiography revealed a normal neoaortic valve with mild aortic regurgitation. The pulmonary homograft was calcified, and there were normal coronary arteries. The patient was prescribed digoxin 250 µg daily with some symptomatic improvement.
A presyncopal episode occurred 6 years later and was associated with further palpitation. On 24-hour ECG recording, nonsustained ventricular tachycardia (VT) was present. Atenolol was prescribed, but the patient continued to experience intermittent palpitations without further presyncope.
The patient was reviewed on an annual basis and remained asymptomatic apart from occasional palpitations. During the last consultation he complained of a mild reduction in exercise tolerance.
Comments: In August 1967 Donald Ross in London performed the first successful procedure to replace a diseased aortic valve with a pulmonary autograft (the patient’s own harvested pulmonary valve), while placing a human homograft valve in the pulmonary position. The pulmonary autograft has been shown to be resistant to long-term degeneration, showing high and stable survival rates.
It is important that any congenital heart disease patient with palpitations and/or documented arrhythmia undergo a thorough hemodynamic assessment. Often arrhythmia can be the first sign of underlying hemodynamic abnormalities that need to be addressed. Hence, the angiographic study performed initially was done bearing this in mind.
CURRENT SYMPTOMS
The patient is able to walk up one flight of stairs but finds more than this difficult and slows due to dyspnea. He is able to walk his dog on flat ground at a measured pace.
NYHA class: II
Comments: Although subjective assessment of symptoms can often be difficult in patients with congenital heart disease, this patient had previously been extremely fit as a soldier, and was hence more aware of his own exertional limitation and concerned about his recent physical decline.
PHYSICAL EXAMINATION
BP 155/75 mm Hg, HR 72 bpm, oxygen saturation 97%
Height 180 cm, weight 102 kg, BSA 2.26 m 2
Surgical scars: Median sternotomy
Neck veins: JVP was not elevated, with normal waveform.
Lungs/chest: Chest was clear.
Heart: The patient was in sinus rhythm. There was a right ventricular heave and a soft pulmonary second sound. There was a grade 2 ejection systolic murmur in the pulmonary area.
Abdomen: Unremarkable
Extremities: There was no pitting edema or clubbing.
LABORATORY DATA
| Hemoglobin | 15.9 g/dL (13.0–17.0) |
| Hematocrit/PCV | 46% (41–51) |
| MCV | 90 fL (83–99) |
| Platelet count | 262 × 10 9 /L (150–400) |
| Sodium | 137 mmol/L (134–145) |
| Potassium | 3.9 mmol/L (3.5–5.2) |
| Creatinine | 0.97 mg/dL (0.6–1.2) |
| Blood urea nitrogen | 5.8 mg/dL (6–24) |
OTHER RELEVANT LAB RESULTS
| Corrected calcium | 2.24 mmol/L (2.20–2.62) |
| Magnesium | 0.8 mmol/L (0.7–1.0) |
Comments: The serum potassium and magnesium are within the normal range. Electrolyte imbalance is an important cause of VT and needs to be excluded.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 72 bpm
PR interval: 205 msec
QRS axis: +74°
QRS duration: 106 msec
QTC duration: 410 msec
Normal sinus rhythm with normal axis and PR/QT intervals. Normal ECG.
Comments: There is no evidence of a long QT syndrome or pre-excitation to explain the palpitation. Atrial arrhythmias in the setting of pre-excitation may lead to broad complex tachycardia.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 58%
The cardiac silhouette was enlarged. There was mild dilatation of the main pulmonary trunk.
Comments: No obvious calcification of the pulmonary homograft is seen (30 years after its original implantation). When severe RVOT obstruction is present, RA and RV enlargement are common. The latter is better seen on a lateral film, not available for this patient.
Also note the lack of dilatation of the ascending aorta.
EXERCISE TESTING
| Exercise protocol: | Modified Bruce |
|---|---|
| Duration (min:sec): | 9:50 |
| Reason for stopping: | Dizziness |
| ECG changes: | Ventricular tachycardia |
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