Petra Jenkins

Urgent resection of a giant left atrial appendage aneurysm and mitral valve replacement in a complex case of Hurler-Scheie syndrome.

Hurler-Scheie syndrome is a rare lysosomal storage disease affecting the cardiovascular system. Besides the cardiac manifestations, it presents with complications from abnormal proteoglycan deposition in soft tissues in many locations, resulting in joint contractures, paraplegia, impaired vision, airway narrowing and restrictive lung function, to name a few. There are very few reports of surgical management of valvular heart disease due to mucopolysaccharidosis (MPS). We describe the successful management of a patient with an extremely challenging case of mitral valve stenosis and a giant left atrial appendage aneurysm due to MPS type 1 (Hurler-Scheie syndrome). The patient underwent mitral valve replacement and excision of the giant left atrial appendage aneurysm; a similar case has not been previously reported.

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation

BACKGROUND Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS We retrospectively reviewed the medical records from two tertiary institutions. RESULTS We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2 was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P>0.05). CONCLUSIONS Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Systolic Anterior Motion Obstructing the Pulmonary Outflow Tract After Tricuspid Valve Replacement

A patient with congenitally corrected transposition of the great arteries who presented with shortness of breath was found to have severe tricuspid regurgitation and right ventricular impairment. After uneventful mechanical systemic tricuspid atrioventricular valve replacement, the patient was extubated within 12 hours. On the first postoperative day, he developed episodes of profound hypotension lasting a few seconds. A transesophageal echocardiogram demonstrated displacement of the interventricular septum that caused systolic anterior motion of the mitral valve into the subpulmonic left ventricle. Mitral valve replacement resolved this complication, and the patient proceeded to do well at discharge.

Intracardiac extension of uterine leiomyomatosis.

A 55-year-old previously healthy woman noticed progressive breathlessness over 6 months. Contrast CT and transthoracic echocardiogram (TTE) showed pulmonary nodules, tricuspid regurgitation and a non-occlusive mass in the right ventricular outflow tract. Pelvic CT contrast scan showed endometrial abnormalities without intravascular invasion. Endometrial fine-needle aspirations and percutaneous lung biopsies yielded no definitive diagnosis. The patient underwent cardiac MRI 2 days prior to cardiac surgery, showing high-contrast uptake on delayed enhancement in the …