Quadricuspid Pulmonic Valve, Pulmonary Artery Aneurysm, and Apical Hypertrophic Cardiomyopathy
A Rare Combination
A 67-year-old man presented with complaints of intermittent episodes of mild exertional dyspnea and substernal chest pain that improved by resting. He had a history of essential hypertension, cigarette smoking, moderate congenital pulmonic stenosis, and severe pulmonary artery dilatation. Physical examination showed a grade 3 systolic murmur in the apex and left upper sternal border, without other anomalies. His diagnostic work-up included an ECG that demonstrated left ventricular hypertrophy and inverted T waves in the anterolateral leads (particularly in leads V4 and V5), as well as a stress echocardiogram without evidence of ischemia and with moderate-to-severe thickening of mid-to-apical left ventricular segments, consistent with apical hypertrophic cardiomyopathy. For further evaluation, he was referred for cardiac magnetic resonance.
The cardiac magnetic resonance revealed normal biventricular size and systolic function, moderate hypertrophy of the apical segments (≤1.5 cm, ratio of apical to basal left ventricular wall thicknesses of 1.9; Figure 1), and no evidence of scar on delayed postcontrast imaging. On 3D magnetic resonance angiography, the main pulmonary and the left pulmonary arteries were aneurysmal (6.1 and 4.0 cm, respectively) and the right pulmonary artery was also dilated measuring 3.4 cm (Figure 2; Video II in Data Supplement). Furthermore, a quadricuspid pulmonic valve was found with moderate regurgitation (regurgitant fraction, 16%) and probably mild stenosis (maximum peak velocity of 1.6 m/s on flow imaging; Figure 3; Video I in Data Supplement).
Because the patient was mildly symptomatic and surgical indications for pulmonary aneurysms are not well established, the patient was managed conservatively with planned serial follow-up.
Quadricuspid pulmonic valve is a rare congenital abnormality, and because of its difficult noninvasive assessment, it is usually discovered in autopsies (reported prevalence in postmortem specimens ranges from 1 in 400 to 1 in 1000).1 It most often presents as an isolated incidental finding, and only few cases of quadricuspid pulmonic valve in combination with pulmonary artery aneurysm have been reported previously.2,3 Although the aneurysm may be partly related to the presence of pulmonary valve stenosis, given its mild severity, we think it may reflect concomitant arteriopathy similar to that associated to bicuspid aortic and pulmonary valves.4 Apical hypertrophic cardiomyopathy may also be difficult to diagnose by transthoracic echocardiography because of the limited visualization of the apex with this technique.
To the best of our knowledge, the association of apical hypertrophic cardiomyopathy quadricuspid pulmonic valve and pulmonary artery aneurysm has never been reported. This case shows the utility of cardiac magnetic resonance for detailed noninvasive depiction of these 3 findings.
The Data Supplement is available at http://circimaging.ahajournals.org/lookup/suppl/doi:10.1161/CIRCIMAGING.118.007698/-/DC1.
- © 2018 American Heart Association, Inc.
- Nollen GJ,
- Kodde J,
- Beek AM,
- Res JC,
- van Rossum AC
- Jamis-Dow CA,
- Barbier GH,
- Watkins MP,
- Lanza GM,
- Caruthers SD,
- Wickline SA