Akiva Tamir

Save a child’s heart: we can and we should

BACKGROUND Congenital heart disease (CHD) causes the death of thousands of children in developing countries. At the Wolfson Medical Center (WMC), a prototype program has been developed to address this issue. METHODS Since 1996, indigent children have been referred to the program, with the cooperation of partners in developing countries. The projects aims are to (a) train their medical personnel at WMC, (b) travel to participating countries to teach, evaluate patients, operate, and promote the development of local centers, and (c) treat children with CHD, at WMC, who lack a local option for care either due to prohibitive costs or unavailability. The projects personnel are state employees who volunteer to treat additional patients within the framework of their salaries, and community volunteers. RESULTS The program has seven partner sites in six countries, including two provinces in China (Hebei and Gansu), Ethiopia, Moldova, Nigeria, the Palestinian Authority, and Tanzania. Five physicians and 10 nurses have been trained from five participating countries. Over the past 4 years, 11 teaching trips have been made abroad, and operations have been performed at four partner sites. A total of 386 patients have been operated on-360 at WMC and 26 at other sites. There have been 17 (4.3%) acute deaths. Follow-up is 92% complete with 3 late deaths reported. CONCLUSIONS Hospital-based regional centers can be created to promote the care of children with CHD in developing countries. Good results and follow-up care can be provided with appropriate planning.

Inverted left atrial appendage presenting as a left atrial mass after cardiac surgery

Inversion of the left atrial appendage can masquerade as a new left atrial mass. Failure to be aware of this entity can result in unnecessary diagnostic and therapeutic procedures. If the entity is diagnosed intraoperatively, treatment is simple with external reduction and ligation.

Right ventricular outflow tract strategies for repair of tetralogy of Fallot: effect of monocusp valve reconstruction.

OBJECTIVES The absence of a pulmonary valve (PV) after tetralogy of Fallot (TOF) repair has been shown to impact postoperative right ventricular (RV) function. The purposes of this study were to (i) compare early outcomes after PV-sparing vs transannular patching (TAP) with monocusp valve reconstruction or TAP alone and (b) assess the mid-term results after polytetrafluoroethylene (PTFE) membrane monocusp reconstruction. METHODS From 2003 to 2009, 163 patients underwent TOF repair. Sixty-nine patients (42.3%) underwent a PV-sparing procedure (Group A), 74 (45.4%) underwent PTFE membrane monocusp valve reconstruction (Group B) and 20 (12.3%) underwent TAP only (Group C). Early outcomes were evaluated by the right-to-left ventricular pressure ratio, RV outflow tract gradient, tricuspid and PV function, intensive care unit (ICU) parameters and need for reintervention. Group B patients were also evaluated at intermediate term for clinical and echocardiographic parameters, including tricuspid and monocusp valve function and mobility. RESULTS The median age, weight and PV Z-value of Group B patients were significantly lower; 20.5 months, 9.3 kg and -4, respectively. Postoperatively, the right-to-left ventricular pressure ratio was <0.5 in all groups. Mechanical ventilation time, fluid drainage duration and total ICU stay showed no significant difference between Groups A and B, while Group C was significantly longer (P < 0.01). There were five (3%) early deaths: three from Group A and two from Group B. The incidences of moderate or severe pulmonary insufficiency (PI) on discharge were 8.2% in Group A, 9% in Group B and 50% in Group C (P < 0.001). Among Group B patients, 85% of the evaluated patients had less than moderate PI in the intermediate-term follow-up, QRS duration <140 ms in 83.3% and right-to-left ventricular diameter ratio of 0.6 ± 0.2. Two (2.6%) patients underwent reoperation for monocusp replacement. There were two (2.7%) mid-term deaths. CONCLUSIONS The use of a PTFE membrane monocusp valve and a valve-sparing strategy prevents immediate PI and improves short-term clinical outcomes. PTFE membrane monocusp appears advantageous in preventing severe intermediate-term PI and facilitates the preservation of RV function.

Ruptured sinus of Valsalva aneurysm complicated by myocardial ischemia: pathogenetic mechanisms

Significant left-to-right shunt in combination with severe aortic regurgitation (AR) accelerates the development of symptoms after rupture of congenital sinus of Valsalva aneurysm (SVA) in spite of intact coronary arteries. We depict a rare description of a situation where acute coronary syndrome was the first manifestation of such an occurrence. We believe that the progress of the myocardial ischemia after ruptured SVA depends on the severity of AR and the quantity of the left-to-right shunt. Prompt recognition and surgical repair are indicated to prevent complications of myocardial infarction.

Massive pulmonary emboli in children: does fiber-optic-guided embolectomy have a role? Review of the literature and report of two cases.

Massive pulmonary emboli is a rare disease in children, with only 39 reported cases in the last 50 years. Almost 50% of the patients died suddenly without receiving medical treatment. Most of the patients who were managed medically (70% of the treated patients) underwent surgical pulmonary embolectomy with 80% survival. Surgical pulmonary embolectomy is a blind procedure that can be improved by using intraoperative angioscopy. This technique was reported in adults with good results. In this article, we describe two pediatric patients who underwent fiber-optic-guided surgical pulmonary embolectomy. To our knowledge, this technique has never been reported in the pediatric population.

OP09.08: Characterisation of subtypes of isolated ventricular septal defects demonstrated by fetal echocardiography and their postnatal outcome

Objectives: To assess the performance of first trimester ultrasound in identifying isolated major congenital heart defects (CHD) in fetuses with normal anatomy and normal nuchal translucency (NT). Methods: This was a retrospective two-centre study of 15778 pregnant women examined at 11 to 13+6 gestational weeks. Women were examined using 4-chambers and 3-vessels views of the fetal heart both in 2D mode and High Definition (HD) Power Flow Doppler mode. Results: A total of 15778 patients were included in the study. The median maternal age was 29 years. The median Crown–rump length was 67 mm (range, 45-84 mm). Overall, there were 211 cases with CHD. Only 3 major CHD was missed at the time of first trimester scan (1 TOF, 1 TGA, 1 inlet VSD). 10 cases were lost to follow up and were excluded from the study. Among remaining 201 fetuses with CHD 80 fetuses (39,8%) had chromosomal abnormalities and 60 fetuses (29,8%) had congenital abnormalities of other anatomical structures. 61 fetuses (30,3%) did not have chromosomal abnormalities or other anatomical malformations. In 20 fetuses (9,9%) the only additional finding was NT above 99 centile, in 10 fetuses (4,9%) NT ranged from 95 to 99 centile. 13 fetuses (6,5%) showed normal NT but reversed flow in ductus venosus or tricuspid regurgitation. 18 fetuses (9%) CHD was the only finding. Among 3 fetuses with CHD missed in the first trimester in 1 fetus there were no abnormal findings and TOF was diagnosed at 20 weeks of pregnancy. In other 2 fetuses the NT was increased (3,3 mm and 5,1 mm), but the correct diagnosis (TOF and inlet VSD, respectively) was made at 20 weeks only. Conclusions: The vast majority of major CHD can be diagnosed at the time of 1st trimester ultrasound screening. Up to 9% of fetuses with major CHD do not have any other anatomical malformation, abnormal karyotype, increased NT or abnormal Doppler markers and should actively be looked for in every fetus undergoing first-trimester ultrasound.

P06.03: Measurement of the aortic outlet angle by ultrasound in fetuses with and without congenital heart malformations involving the left ventricular outflow tract

Objectives: The angle between the aortic outflow and the inter-ventricular septum can be measured during sonographic fetal survey while viewing the left ventricular outflow tract (LVOT angle). Our aim was to compare the LVOT angle between fetuses with and without congenital heart malformations involving the LVOT. Methods: In this prospective cross-sectional study we measured the LVOT angle in fetuses with and without conotruncal anomalies. At the level of ‘‘4 chamber view’’ the transducer was tilted so that the LVOT was demonstrated, creating a ‘‘5 chamber view’’. The LVOT angle was measured during early systole at complete opening of the aortic valve, from the interventricular septum to the base of the aortic valve. Results: LVOT angle measurements were acquired from 299 fetuses at 15-38 weeks. LVOT angle were between 127-163 degrees, mean 148.2, independent of gestational age. The LVOT angle was significantly higher in fetuses with TGA or AS, compared with fetuses without heart malformations involving the LVOT (165.0 vs. 148.2, p<0.001). The LVOT angle was significantly lower in fetuses with AVC, compared with fetuses without heart malformations involving the LVOT (125.5 vs. 148.2, p<0.001). Conclusions: The LVOT angle does not change during pregnancy. The LVOT angle significantly differs between fetuses with and without heart malformations involving the LVOT.

Selected ReportsMassive Pulmonary Emboli in Children: Does Fiber-optic-Guided Embolectomy Have a Role? Review of the Literature and Report of Two Cases

Massive pulmonary emboli is a rare disease in children, with only 39 reported cases in the last 50 years. Almost 50% of the patients died suddenly without receiving medical treatment. Most of the patients who were managed medically (70% of the treated patients) underwent surgical pulmonary embolectomy with 80% survival. Surgical pulmonary embolectomy is a blind procedure that can be improved by using intraoperative angioscopy. This technique was reported in adults with good results. In this article, we describe two pediatric patients who underwent fiber-optic-guided surgical pulmonary embolectomy. To our knowledge, this technique has never been reported in the pediatric population.