The patient was a 4-year, 1-month old boy who presented with a postnatal diagnosis of a constellation of left-sided lesions, including coarctation of the aorta, transverse arch hypoplasia, bicuspid aortic valve, mitral stenosis, and a borderline-sized left ventricle.
At the time of birth, due to questions about the adequacy of the left ventricle to support a biventricular circulation, a cardiac MRI was obtained to assess the left ventricular volume. Volume was found to be 23 ml/m2 with a left ventricular ejection fraction of 55%. The transverse aortic arch was hypoplastic with a diameter between 3.5 and 4.5 mm. Based on these MRI findings, the decision was made to proceed with coarctation repair via a left posterolateral thoracotomy.
At two weeks of age, the 3.19 kg baby underwent the coarctation repair with extended end-to-end anastomosis with good result. Postoperatively, there was a minimal residual gradient (approximately 8 mm Hg) between upper and lower extremities. Convalescence was unremarkable and was discharged shortly thereafter.
Over the course of the next six months, the patient developed worsening valvar aortic stenosis (gradient of 75-80 mm Hg) and was referred for balloon aortic valvuloplasty. At the time of catheterization, the aortic valve annulus was measured at 8.7 mm. The aortic valve underwent balloon angioplasty with an 8 mm Tyshak II balloon with a residual gradient of approximately 30-35 mm Hg. Other notable findings at the time of catheterization were mild residual gradient across aortic arch repair (approximately 5-6 mm Hg postvalvuloplasty), mild mitral stenosis, elevated pulmonary artery pressures (mean of approximately 48-50 mm Hg), elevated transpulmonary gradient, and left ventricular diastolic dysfunction with an elevated LVEDP (22 mm Hg at baseline, down to 17 mm Hg post-balloon valvuloplasty).
The patient re-presented to the hospital at 15 months of age with recurrent valvar aortic stenosis (gradient of 50-55 mm Hg) and a new finding of a subaortic membrane. At the time of catheterization, the aortic valve annulus was measured at 9.5 mm. The aortic valve underwent balloon angioplasty with a 10 mm Tyshak II balloon with a residual gradient of approximately 18-20 mm Hg. The patient still had an increased LVEDP of approximately 18 mm Hg at the time. Other notable findings at time of catheterization were resolution of mitral stenosis, elevated pulmonary artery pressures (mean of approximately 28-30 mm Hg, thought to be secondary to increased LVEDP), and resolution of residual gradient across aortic arch (0-3 mm Hg).
The patient was followed at an outpatient and did well until four years of age. Subsequent echocardiograms demonstrated worsening left ventricular outflow tract obstruction with a peak gradient of 67 mm Hg and moderate aortic valve regurgitation. By echocardiogram, the aortic annulus measured 11 mm with preservation of left ventricular function. The patient was presented at a case management conference and was put forward for surgical intervention on the left ventricular outflow tract. Given his size and clinical course, the decision was made to proceed with the Ross procedure.
The patient then underwent the Ross-Konno procedure with 19 mm pulmonary homograft, resection of subaortic membrane, and septal myectomy. The post repair transesophageal echocardiogram demonstrated no residual left ventricular outflow tract obstruction, no aortic insufficiency, good biventricular function, and an unobstructed right ventricular outflow tract.
The patient’s postoperative convalescence was unremarkable. The patient was extubated and transitioned to full diet on postoperative day one, chest tubes removed on postoperative day two, and the patient was discharged home on postoperative day four. The patient is currently doing well as an outpatient without readmission.