Median Arcuate Ligament Syndrome with Orthostatic Intolerance: Intermediate-Term Outcomes following Surgical Intervention.

OBJECTIVES: To report the intermediate-term outcome following surgical intervention for median arcuate ligament syndrome (MALS) in adolescents and young adults with orthostatic intolerance (OI) to assess clinical improvement in the gastrointestinal and 5 other functional domains and if relief of arterial obstruction is associated with resolution of clinical symptoms. STUDY DESIGN: Thirty-one patients were given 2 dysautonomia-designed questionnaires to assess changes in symptoms following operative intervention in 6 functional domains and underwent postoperative repeat abdominal ultrasound examinations. RESULTS: Average follow-up after surgery was 22.4 ± 14.8 months. Self-assessed quality of health on a Likert scale (1-10 with 10 being normal) improved from 4.5 ± 2.1 preoperatively to 5.3 ± 2.4 postoperatively (P = not significant). Gastrointestinal symptoms of abdominal pain, nausea, and vomiting improved in 63% (P = .007), 53% (P = .040), and 62% (P = .014) of patients, respectively. Cardiovascular symptoms of dizziness, syncope, chest pain, and palpitations improved in 45% (P = not significant), 50% (P = not significant), 54% (P = .043), and 54% (P = .037) of patients, respectively. Transabdominal ultrasound peak supine expiratory velocity decreased from 348 ± 105 cm/s preoperatively to 251 ± 109 cm/s at 6 months or more after a ligament release procedure. Decrease of the postoperative celiac artery Doppler velocity was not associated with an improvement in gastrointestinal symptoms (P = .075). CONCLUSIONS: Adolescent and young adult patients with median arcuate ligament syndrome and OI have a good response to surgical intervention. About two-thirds of patients report significant improvement in symptoms of abdominal pain, nausea, and vomiting. Despite these encouraging data, many patients with MALS and OI continue to have an impaired quality of health.

Sinus Node Sparing Novel Hybrid Approach for Treatment of Inappropriate Sinus Tachycardia/Postural Orthostatic Sinus Tachycardia With New Electrophysiological Finding.

The ideal treatment of Inappropriate Sinus Tachycardia (IST) and Postural Orthostatic Tachycardia Syndrome (POTS) still needs to be defined. Medical treatment yields suboptimal results, endocardial ablation of the sinus node (SN) may risk phrenic nerve damage and open heart surgery may be accompanied by unjustified invasive risks. We describe our first experience of 50 consecutive patients (41 females, 22.83 ± 3.91 years) having undergone a novel hybrid thoracoscopic ablation for drug resistant IST (n = 39, 78%) or POTS (n = 11, 22%). The SN was identified with the help of 3D mapping. Surgery was performed through 3 (5 mm) ports from the right side. A minimally invasive approach with a radio frequency bipolar clamp was utilized to a new target sparing the SN region, to isolate the superior and the inferior caval veins, and a crista terminalis line was made. All lines were interconnected. Normal SR was restored in all patients at the end of the procedure. All patients discontinued medication during the follow-up. After a blanking period of 6 months all patients presented stable SR. At a mean of 28.4 ± 1.2 months, normal SN ruction and chronotropic response to exercise was present. In the 11 patients initially diagnosed with POTS, no syncope occurred. During the follow-up, pericarditis was the most common complication (39 patients; 78%) with complete resolution in all cases. In conclusions the preliminary results of our first experience with a SN sparing novel hybrid ablation of IST/POTS, using surgical thoracoscopic video-assisted epicardial ablation combined with concomitant endocardial 3D mapping may prove an efficient and safe therapeutic option in patients with symptomatic drug resistant IST and POTS. Importantly, in our study all patients had a complete resolution of the symptoms and restored normal SN activity.