Factors associated with perioperative mortality in children and adolescents operated for tetralogy of Fallot: A sub-Saharan experience.

BACKGROUND: Patients with tetralogy of Fallot are now surviving to adulthood with timely surgical intervention. However, many patients in low-income countries have no access to surgical intervention. This paper reports the surgical access and perioperative mortality in a sub-Saharan center that was mainly dependent on visiting teams. METHODS: We reviewed records of patients operated from January 2009 to December 2014. We examined perioperative outcomes, primarily focusing on factors associated with perioperative mortality. RESULTS: During this period, 62 patients underwent surgery. Fifty-seven (91.9%) underwent primary repair, while 5 (6.5%) underwent palliative shunt surgery. Of the five patients with shunt surgery, four ultimately underwent total repair. Eight (12.9%) patients died during the perioperative period. Factors associated with perioperative mortality include repeated preoperative phlebotomy procedures (P < .001), repeated runs and long cardiopulmonary bypass time (P < .001), and aortic cross-clamp time (P < .001), narrow pulmonary artery (PA) valve annulus diameter (P = .022), narrow distal main PA diameter (P = .039), narrow left branch PA diameter (P = .049), and narrow right PA diameter (P = .039). Of these factors, cardiopulmonary bypass time/aortic cross-clamp time and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality. CONCLUSION: In this series of consecutive patients operated by a variety of humanitarian surgical teams, cardiopulmonary bypass time/aortic cross-clamp time, and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality risk. As some of these factors are modifiable, we suggest that they should be considered during patient selection and at the time of surgical intervention.

Hydatid cyst of the interventricular septum.

While cardiac involvement is not a common presentation in human echinococcosis, it may lead to life-threatening complications including cyst rupture; anaphylactic shock; tamponade; pulmonary, cerebral or peripheral arterial embolism; acute coronary syndrome; dysrhythmias; infection; ventricular or valvular dysfunction, as well as sudden death. Here we report a 9-year old girl who was diagnosed to have hydatid cyst of the interventricular septum four years after diagnosis and medical treatment of pulmonary hydatidosis. Presentation, management and follow-up of the patient is discussed.

Outcome in Children Operated for Membranous Subaortic Stenosis: Membrane Resection Plus Aggressive Septal Myectomy Versus Membrane Resection Alone.

BACKGROUND: The optimal surgical procedure for treatment of fibromembranous subaortic stenosis has been a subject of debate. We report our experience with patients treated for membranous subaortic stenosis using membrane resection alone and membrane resection plus aggressive septal myectomy. METHODS: Patients followed in the pediatric cardiology clinic of a university hospital, who had undergone surgery for subaortic stenosis between 2002 and 2013 were reviewed. Recurrence of subaortic membrane, residual left ventricular outflow gradient, and aortic valve function were analyzed. RESULTS: Forty-six patients underwent surgery for subaortic membrane. Of these, 19 had membrane resection plus aggressive septal myectomy, while 27 had membrane resection alone. Mean age at surgery for the membrane resection group was 7.7 ± 3.9 years and 10.9 ± 3.6 years for the membrane resection plus aggressive myectomy group. Preoperative subaortic gradient for the membrane resection group was 75.5 ± 26.7 mm Hg and 103.2 ± 39.7 mm Hg for the membrane resection plus aggressive myectomy group. The mean follow-up left ventricular outflow tract gradient was 42.3 ± 31.3 mm Hg in the membrane resection group, while it was 11.6 ± 6.3 mm Hg in the aggressive septal myectomy group. Nine patients from the membrane resection group had significant regrowth of the subaortic membrane during the follow-up period, while none of the aggressive septal myectomy group had detectable membrane on echocardiography. Seven of the nine patients with recurrence of the subaortic membrane underwent subsequent membrane resection plus aggressive septal myectomy. Intraoperative finding in all these redo cases was recurrence (growth) of a subaortic membrane. CONCLUSION: Aggressive septal myectomy offers less chance of recurrence, freedom from reoperation, and an improved aortic valve function. This is especially important in sub-Saharan settings where a chance of getting a second surgery is unpredictable.

Pulmonary artery aneurysm with patent arterial duct: resection of aneurysm and ductal division.

Congenital or acquired aneurysm of the pulmonary artery (PA) is rare. Although aneurysms are described following surgical treatment of patent ductus arteriosus (PDA), occurrence of this lesion in association with PDA without previous surgery is extremely uncommon. An eight-year-old patient with PDA and aneurysm of the main PA is described in this report. Clinical diagnosis of PDA was made upon presentation. Diagnosis of PA aneurysm was suspected on chest x-ray and was confirmed on transthoracic echocardiography. Successful surgical resection of the aneurysm and division of the duct were performed under cardiopulmonary bypass. The patient did well on follow-up both from clinical and echocardiographic point of view.

Straightened sternal wire causes iatrogenic pectus carinatum after cardiac surgery.

Pectus carinatum is a protrusion deformity of the anterior chest wall that is most likely caused by a disproportionate growth of the costal cartilages compared with the remainder of the thoracic skeleton. A young boy had previously undergone corrective congenital heart operation, after which a prominent sternal protrusion was noted. During the past year, the protrusion had greatly increased in size and had become recurrently infected. Chest X-ray showed that a sternal wire, the ends of which were pointing toward the skin, had straightened. Operative intervention included removal of the offending wire, draining a chronic abscess, and shaving the protruding sternum so that it conformed to the rest of the sternum.

Improved current era outcomes in patients with heterotaxy syndromes.

OBJECTIVE: Patients with heterotaxy syndrome have a myriad of visceral and cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current era management strategies have improved outcomes in patients with visceral heterotaxy. METHODS: A retrospective review (1994-2008) of our database identified 45 consecutive heterotaxy patients who underwent surgical palliation. There were 29 patients with right atrial isomerism (RAI) and 16 patients with left atrial isomerism (LAI). Functional single ventricle was present in 32 patients. Pulmonary outflow obstruction was present in 29 of the patients. Twenty patients had total anomalous pulmonary venous return (TAPVR), of which 9 were obstructed. An initial neonatal surgical approach was performed in 27 patients. Thirty patients had systemic to pulmonary artery shunt. Mean follow-up was 43.6+/-47 months in RAI and 41.0+/-40.8 months in LAI patients (p=0.4). RESULTS: There were three hospital deaths, all after the first operation, and four interstage deaths (six RAI; one LAI). There were no deaths after cavopulmonary shunt, Kawashima or Fontan operation. A multivariate Cox regression identified greater than moderate atrioventricular valve regurgitation (Hazard Ratio (HR) 17.5, p=0.017) and obstructed TAPVR (HR 17.5, p=0.007) as factors associated with increased RAI mortality. Due to the absence of late mortality in both groups, patient survival at 3 years were 79% in RAI and 94% in LAI patients and remained stable after that (p=0.22). All survivors but one are in NYHA class I or II, without significant cardiovascular related symptoms. LAI patients have a higher incidence of sinus node dysfunction than RAI patients (47% vs 12.5%, p=0.009). CONCLUSIONS: Surgical outcomes in heterotaxy patients are improving in the current era. The risk for operative mortality and attrition is highest between the first and second stage palliation procedures. Significant atrioventricular valve regurgitation and obstructed TAPVR remain risk factors for RAI mortality. Survivors are doing well with no activity restrictions, although LAI patients maintain a higher proclivity of sinus node dysfunction.