Successful repair of neonatal tricuspid regurgitation due to chordae rupture

Abstract

IntroductionFunctional tricuspid regurgitation (TR) is transient and benign in stressed infants. In contrast, severe TR caused by a flail leaflet is a rare cause of neonatal cyanosis. The outcome may be fatal if not recognized early (1,2). We report on a full-term female neonate presented with cyanosis caused by severe TR due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion for keeping ductal patency, successful early repair was achieved with polytetrafluoroethylene artificial chordae implantation. Early diagnosis and timely surgery can be lifesaving. Case presentationA 3,044-gm full term female was born by Cesarean section because of fetal distress and meconium stain. The first and fifth minutes Apgar score was 5 and 9. On physical examination, a grade III/VI pansystolic murmur was noted diffusely. She was cyanotic and the oxygen saturation was 85%. A two-dimensional echocardiography showed severe TR due to a flail and thickened anterior leaflet (Figure 1A,B) with the calculated tricuspid Z score of 1.28, and large patent ductus arteriosus. The antegrade pulmonary flow was compromised and a right-to-left shunt through the patent foramen ovale was noted. The hemodynamic characteristics were quite similar to that of the pulmonary atresia with intact ventricular septum.At 6-day-old, cyanosis got worse due to impending ductal closure. She was intubated and prostaglandin E1 was infused (0.2 µg/kg/min), then the condition was stabilized and surgery was carried. Under mild hypothermia with cold crystalloid cardioplegic cardiac arrest, the patent ductus was ligated and the right atrium was opened. A flail tricuspid anterior leaflet due to extensive marginal chordae rupture was confirmed. The papillary muscle showed no ischemic change. The length of the septal leaflet chordae was measured as the reference and two loops of 6-0 expanded polytetrafluoroethylene artificial chordae were implanted over the unsupported margin of the anterior leaflet. A tricuspid annuloplasty was not necessary because the annulus was not dilated. The patent foramen ovale was not closed for pop-off purpose. Nitric oxide was not needed and under minimal inotrope support, the patient was recovered uneventfully. She was extubated 7 days after the operation Abstract: Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.

Mid-Term Follow-Up of Neonatal Neochordal Reconstruction of Tricuspid Valve for Perinatal Chordal Rupture Causing Severe Tricuspid Valve Regurgitation

“…Preoperative stabilization of this subset may require PGE1, diuretics, inotropic support, mechanical ventilation, inhaled nitric oxide, sildenafil, and even extracorporeal membrane oxygenation (ECMO). 5 -7,9 We were able to stabilize all our patients without need for inhaled nitric oxide or ECMO support. Antenatal or early postnatal diagnosis of this condition, combined with prompt and planned delivery, aggressive stabilization, and early surgical repair prior to the occurrence of hemodynamic instability and irreversible secondary end-organ damage may improve the chances for a good outcome.…”

“…1 Papillary muscle rupture secondary to myocardial ischemia or infarction may result from antepartum or peripartum asphyxia, premature ductal closure, congenital heart disease, rhesus iso-immunization, congenital endocarditis, thromboembolism, maternal connective tissue disorder, viral infections, traumatic rupture during birth process, or other more obscure causes. 5 -8 Susceptibility to ischemia is higher in the anterior papillary muscle of the tricuspid valve. Factors likely to be responsible for this are (i) poor diastolic coronary perfusion in the presence of high RV pressure, (ii) high oxygen demand of the papillary muscle supporting ATL, and (iii) supply by the distal extreme of coronary circulation.…”

“…Factors likely to be responsible for this are (i) poor diastolic coronary perfusion in the presence of high RV pressure, (ii) high oxygen demand of the papillary muscle supporting ATL, and (iii) supply by the distal extreme of coronary circulation. 7…”

“…Antenatal or early postnatal diagnosis of this condition, combined with prompt and planned delivery, aggressive stabilization, and early surgical repair prior to the occurrence of hemodynamic instability and irreversible secondary end-organ damage may improve the chances for a good outcome. 5 -7,9…”

“…Although the use of artificial chordae in pediatric and neonatal tricuspid valve repair has been described, 1,6,7,9,11,12 only one published series reported midterm outcome of neonatal repair with median follow-up of 33 months. 5…”