Age: 56 years
Gender: Male
Occupation: Attorney
Working diagnosis: Repaired tetralogy of Fallot
HISTORY
At the age of 6 years, this patient had a Brock procedure (1958). Using a transpulmonary approach, Lord Brock resected subpulmonary muscle to relieve the pulmonary stenosis, leaving the patient’s VSD untouched.
Three years later, at the age of 9 years, the patient presented with further cyanosis and fainting, and underwent tetralogy repair, also by Lord Brock. The operative note described the following: “Bicuspid pulmonary valve—stretched with an expanding dilator,” “the fibro-endocardial tissue constituting the infundibular stenosis was carved away liberally,” “the VSD was closed with a pericardial patch.” The postoperative notes document a pulmonary diastolic murmur.
A year later the patient experienced an episode of ventricular tachycardia (VT) requiring direct current (DC) cardioversion at a local hospital. Again, at age 14 years, he had a further episode of sustained VT, which apparently occurred following a blow to the chest. This resolved spontaneously. The patient felt well and had no further workup or adverse event.
There was no routine cardiac follow-up until the patient was 44 years old. At that time he complained of palpitations. Although each episode was short lived, he had noted these several times over the previous 9 years.
On reassessment he was found to have moderate to severe pulmonary regurgitation by echocardiography. Both sustained VT and nonsustained VT were also documented on 24-hour Holter ECGs over the following 5 years.
At age 50 his exercise capacity was normal, and his only complaint was of occasional palpitations. His pulmonary regurgitation was reevaluated and found to be severe by echocardiography and cardiac catheterization. He had normal coronary arteriograms.
Because the patient was “asymptomatic” with above-average exercise tolerance, a decision was made to defer pulmonary valve replacement and that there was no current indication for electrophysiologic ablation. Medication for arrhythmia was declined by the patient, who felt that this might treat only the palpitations rather than the underlying cause.
In the years that followed, palpitations were described as either single beats, or short-lived regular and fast heart rhythms relieved by a Valsalva maneuver. His exercise tolerance remained very good.
On the day of presentation, the patient was walking his dog briskly uphill and noted the onset of very fast palpitations and dizziness unrelieved by a Valsalva maneuver. As the rhythm persisted he felt chest pain, diaphoresis, and a feeling like blood was draining from his fingers. He collapsed but did not lose consciousness. A passerby called for help. Paramedics were soon at the scene and gave him aspirin and oxygen. He was taken immediately to a nearby hospital, where the following ECG was obtained.
A DC cardioversion was promptly performed, and sinus rhythm with a stable blood pressure was restored. There were no electrolyte abnormalities, and blood tests did not show any elevation of troponin. He felt well thereafter and was discharged from the local hospital after 24 hours of observation. Follow-up was arranged with a tertiary care center.
The patient was a nonsmoker with no other medical problems. His eldest brother had unspecified congenital heart disease, but there was no history of coronary artery disease in the family.
Comments: Historically, pulmonary regurgitation was thought to be a benign lesion. Pulmonary regurgitation is usually the dominant lesion in patients who have had tetralogy repair. A severe degree of pulmonary regurgitation was more likely if a transannular patch was used in the repair (not used in this patient). The diastolic murmur was likely due to pulmonary regurgitation.
The blow to the chest may have resulted in mechanical energy being converted to electrical energy, which resulted in an arrhythmia.
The decision to replace the pulmonary valve after repair of TOF is one of the most commonly discussed and controversial issues in this field. The decision to wait was based on his normal RV function, very good exercise tolerance, and absence of symptoms.
The rhythm is VT. The regular, broad complex tachycardia (rate of 214 bpm), with LBBB conduction pattern and superiorly directed QRS axis is consistent with VT.
PHYSICAL EXAMINATION
BP 120/60 mm Hg, HR 60 bpm, oxygen saturation (right hand) 100%
Height 188 cm, weight 70 kg, BSA 1.91 m 2
Surgical scars: Midline sternotomy scar
Neck veins: JVP was not elevated, and the waveform was normal.
Lungs/chest: Chest was clear to auscultation.
Heart: The heart rhythm was regular. There was an RV heave. Auscultation revealed a normal first heart sound, an ejection systolic murmur followed by a single second heart sound, and a low-pitched early diastolic murmur at the left sternal edge, which was accentuated by inspiration. Peripheral pulses were palpable and equal. Additionally, a high-pitched early diastolic murmur at the right sternal edge was heard, felt to reflect aortic regurgitation.
Abdomen: No abnormality detected
Extremities: There was no clubbing or dependent edema.
Comments: Significant JVP elevation in a patient with repaired tetralogy usually reflects problems requiring reoperation. The absence of an abnormal V-wave suggests there is not substantial tricuspid regurgitation. When tricuspid regurgitation occurs in such patients, it is usually because of RV systolic dysfunction.
An RV heave in such a patient without RVOT obstruction indicates that pulmonary regurgitation is moderate to severe. The absence of such a lift may reflect mild hemodynamic problems or be due to physical factors or lack of discernment on the part of the examiner.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 66 bpm
QRS axis: +115°
QRS duration: 137 msec
Sinus rhythm with an RBBB. QRS axis: Initial forces are at +115°, with a terminal RBBB. Six months previously the QRS duration had been 160 msec, and a decade prior it had been 120 msec.
Comments: RBBB was typical in patients with tetralogy repair due to the right ventriculotomy or the placement of the VSD patch. More recently, RBBB is somewhat less common in patients having surgery because a transatrial and transpulmonary approach is now used. QRS duration usually lengthens with time, reflecting increasing RV volume. Increase in QRS duration over time is a marker predictive of sudden cardiac death and may be more sensitive than absolute values.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 49%
Upper normal heart size with prominent main pulmonary artery (PA) and RVOT. The lung markings are normal, and there is evidence of a prior sternotomy. RA prominence. Left aortic arch.
Comments: There is a bulging area ( arrow ) of the left cardiac silhouette that corresponds to a dilated main PA and RVOT.
EXERCISE TESTING
| Current Study | 4 Years Prior | |
|---|---|---|
| Exercise protocol: | Modified Bruce | Modified Bruce |
| Duration (min:sec): | 13:08 | 15:35 |
| Reason for stopping: | Fatigue | Fatigue |
| ECG changes: | None | None |
| Rest | Peak | Peak | |
|---|---|---|---|
| Heart rate (bpm): | 60 | 150 | 155 |
| Percent of age-predicted max HR: | 91 | 92 | |
| O 2 saturation (%): | 100 | 100 | 100 |
| Blood pressure (mm Hg): | 120/60 | 160/80 | 165/80 |
| Peak V o 2 (mL/kg/min): | 30 | 39 | |
| Percent predicted (%): | 75 | 124 | |
| Ve/V co 2 : | 24.7 | 22.1 | |
| Metabolic equivalents: | 9.6 | ||
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