Antonio González-Calle

Surgical technique for heart transplantation: a strategy for congenital heart disease

The standard techniques for orthotopic heart transplantation often require certain adjustments when the procedure is carried out for complex congenital heart disease. This is because of both the unusual anatomy and possible distortions caused by previous surgery. Such technical adjustments have been described in various published reports over the years. Those reports, when combined, do cover the full spectrum of the technical difficulties that may be encountered, whether the defects are in their original form or altered by surgery, such that no cardiac malformation or distortion would prohibit transplantation. However, those reports are comprehensive only when combined. None of the individual reports addresses all the possible technical challenges. Consequently, the available information is somewhat fragmented. In addition, the generic aspect of the described technical strategies is not always given the emphasis that it deserves. Indeed, occasionally a technique may be presented as a specific solution for a specific malformation, without necessarily pointing out that the same technique may be applied to other hearts with different overall pathologies but which share that specific malformation. The aim of this review article was to combine all the available published information in one article in a manner that constructs a simple but comprehensive and generic system of decision-making that may be applied to any heart in order to determine the exact technical adjustments needed for transplantation in each case. Such a strategy is possible for two reasons. First, only a few anatomical sites are technically significant, namely the points of anastomosis between the donors organ and the recipient. The rest of the intracardiac morphology does not affect the operation and may be ignored. Second, each of those anatomical sites can present difficulties in only a few ways, and each of those few difficulties has a well-described and published solution already. Therefore, the exact technical adjustments required in each case may be worked out by the sequential assessment of the anastomotic sites alone.

Three-dimensional printed models for surgical planning of complex congenital heart defects: an international multicentre study

OBJECTIVES To evaluate the impact of 3D printed models (3D models) on surgical planning in complex congenital heart disease (CHD). METHODS A prospective case-crossover study involving 10 international centres and 40 patients with complex CHD (median age 3 years, range 1 month-34 years) was conducted. Magnetic resonance imaging and computed tomography were used to acquire and segment the 3D cardiovascular anatomy. Models were fabricated by fused deposition modelling of polyurethane filament, and dimensions were compared with medical images. Decisions after the evaluation of routine clinical images were compared with those after inspection of the 3D model and intraoperative findings. Subjective satisfaction questionnaire was provided. RESULTS 3D models accurately replicate anatomy with a mean bias of -0.27 ± 0.73 mm. Ninety-six percent of the surgeons agree or strongly agree that 3D models provided better understanding of CHD morphology and improved surgical planning. 3D models changed the surgical decision in 19 of the 40 cases. Consideration of a 3D model refined the planned biventricular repair, achieving an improved surgical correction in 8 cases. In 4 cases initially considered for conservative management or univentricular palliation, inspection of the 3D model enabled successful biventricular repair. CONCLUSIONS 3D models are accurate replicas of the cardiovascular anatomy and improve the understanding of complex CHD. 3D models did not change the surgical decision in most of the cases (21 of 40 cases, 52.5% cases). However, in 19 of the 40 selected complex cases, 3D model helped redefining the surgical approach.

A simple surgical technique to prevent atrial reentrant tachycardia in surgery for congenital heart disease

OBJECTIVES To present and test a simple surgical technique that may prevent atrial reentrant tachycardia following surgery for congenital heart disease. This arrhythmia is one of the commonest long-term complications of such a surgery. It may occur many years (even decades) after the operation. It is usually explained as a late consequence of right atriotomy, which is an inherent component of many operations for congenital heart disease. Right atriotomy results in a long scar on the right atrial myocardium. This scar, as any scar, is a barrier to electrical conduction, and macro-reentrant circuits may form around it, causing reentrant tachycardia. However, this mechanism may be counterchecked and neutralized by our proposed method, which prevents reentrant circuits around right atriotomy scars. METHODS The proposed method is implemented after termination of cardiopulmonary bypass and tying the venous purse-strings. It consists of constructing a full-thickness suture line on the intact right atrial wall from the inferior vena cava (IVC) (a natural conduction barrier) to the atriotomy incision. This suture line is made to cross the venous cannulation sites if these are on the atrial myocardium (rather than being directly on the venae cavae). Thus, the IVC, atriotomy and cannulation sites are connected to each other in series by a full-thickness suture line on the atrial wall. If this suture line becomes a conduction barrier, it would prevent reentrant circuits around right atrial scars. This was tested in 13 adults by electroanatomical mapping. All 13 patients had previously undergone right atriotomy for atrial septal defect closure: 8 of them with the addition of the proposed preventive suture line (treatment group) and 5 without (control group). RESULTS In all 13 cases, the atriotomy scar was identified as a barrier to electrical conduction with electrophysiological evidence of fibrosis (scarring). In the 8 patients with the proposed suture line, this had also become a scar and a complete conduction barrier. In the 5 patients without this suture line, there was free electrical conduction between the IVC and atriotomy scar. CONCLUSIONS The proposed suture line becomes a scar and conduction barrier. Therefore, it would prevent reentrant circuits around atrial scars and their consequent arrhythmias.

Right atrial thrombosis and pulmonary embolism after atrial septal defect repair

A 3-year-old boy underwent surgical closure of a large ostium secundum atrial septal defect. This was complicated with extensive right atrial thrombus formation and pulmonary thromboembolism immediately following surgery. He was managed with emergency surgical thromboembolectomy and anticoagulation. However, new thrombus was formed again immediately. This prompted us to add thrombolysis to his treatment, but with no effect. He died on the fifth postoperative day. A postmortem study confirmed extensive thromboembolism.

Giant Coronary Aneurysms in Kawasaki Disease

La formacion coronaria aneurismatica, con o sin estenosis en el extremo distal de los aneurismas, son caracteristicas bien conocidas de la enfermedad de Kawasaki. Estos aneurismas varian enormemente en gravedad y progresion. Presentamos las imagenes del caso extremo de un nino de 10 anos. El diagnostico de enfermedad de Kawasaki se establecio 2 anos antes del episodio estudiado; entonces se habian identificado aneurismas en la porcion proximal de la arteria coronaria derecha (5 mm) e izquierda (9 mm). Con el tiempo, los aneurismas se agrandaron de manera significativa (20 mm la derecha y 23 mm la izquierda). Ademas, mientras que la arteria coronaria derecha (ACD) se obstruyo, en la arteria descendente anterior (ADA) se desarrollo una estenosis grave, y el paciente sufrio un infarto agudo de miocardio. El estudio angiografico mostro un aneurisma gigante y bloqueo de la ACD y aneurisma gigante y estenosis grave de la ADA. La arteria circunfleja (ACx) tambien mostro una dilatacion aneurismatica significativa, pero sin estenosis (figura 1). Ante esta situacion, se realizo cirugia coronaria (arteria mamaria interna izquierda a la ADA y arteria mamaria interna derecha a la ACD). Las imagenes obtenidas del campo operatorio muestran un aneurisma de la ACD (figura 2A) y de la porcion proximal de la ADA (figura 2B). La evolucion postoperatoria fue favorable y sin eventos adversos. Figura 2. ACD

A simple method of aortic valve reconstruction with fixed pericardium in children

Aortic valve reconstruction with fixed pericardium may occasionally be very useful when treating children with aortic valve disease. This is because diseased aortic valves in children are sometimes too dysmorphic for simple repair without the addition of material, their annulus may be too small for a prosthesis, and the Ross operation may be precluded due to other congenital anomalies such as pulmonary valvar or coronary malformations. Such reconstruction is usually technically demanding and requires much precision. We describe a simple alternative method, which we have carried out in 3 patients, aged 1 week, 3 years and 12 years, respectively, with good early results.

Prevalence and prognostic significance of pulmonary artery aneurysms in adults with congenital heart disease

BACKGROUND Prevalence and prognostic significance of pulmonary artery (PA) dilatation in congenital heart disease (CHD) have never been studied systematically. METHODS Chest X-rays of 1192 consecutive adults with CHD were reviewed. Major diameter of the PA was determined by imaging techniques in those with PA dilatation. A value >29 mm was considered abnormal. Data on anatomy, hemodynamics, residual lesions and outcomes were retrospectively collected. RESULTS Overall prevalence of PA dilatation was 18%. A minority of patients (5.5%) reached 40 mm (aneurysm; PAA) and 1.8% exceeded 50 mm. The most common PAA underlying malformations were pulmonary stenosis (21%), and shunts (55%). Significantly larger diameters were observed in hypertensive shunts (40 mm; IQR 36.7-45 mm vs. 34 mm; IQR 32-36 mm) (p < 0.0001). However, the largest diameters were found in cono-truncal anomalies. There was no significant correlation between PA dimensions and systolic pulmonary pressure (r = -0.196), trans-pulmonary gradient (r = -0.203), pulmonary regurgitation (PR) (r = 0.071) or magnitude of shunt (r = 0.137) (p > 0.05 for all). Over follow-up, 1 sudden death (SD) occurred in one Eisenmenger patient. Complications included coronary (3), recurrent laryngeal nerve (1) and airway (1) compressions, progressive PR (1), and PA thrombosis (1). Coronary compression and SD were strongly associated (univariate analysis) with pulmonary hypertension (120 vs. 55 mm Hg; p = 0.002) but not with extreme PA dilatation (range: 40-65 mm). CONCLUSIONS PA dilatation in CHD is common but only a small percentage of patients have PAA. Clinical impact on outcomes is low. Complications occurred almost exclusively in patients with pulmonary hypertension whereas PA diameter alone was not associated with adverse outcomes.

Aneurismas coronarios gigantes en la enfermedad de Kawasaki

Coronary artery aneurysm, with or without stenosis at the distal end, is a well-recognized characteristic of Kawasaki disease. The aneurysms show considerable variation in severity and progression. We present the imaging findings in a 10-year-old boy with an extreme case of this condition. The diagnosis of Kawasaki disease was established 2 years before the episode presented here. At that time, aneurysms had been detected in the proximal portion of the right (5 mm) and left (9 mm) coronary artery. Over time, the aneurysms had enlarged significantly (to 20 mm and 23 mm, respectively). Furthermore, the right coronary artery (RCA) became obstructed, the left anterior descending artery (LAD) developed severe stenosis, and the patient had an acute myocardial infarction. Angiography study showed a giant aneurysm and occlusion in the RCA, and a giant aneurysm and severe stenosis in the LAD. The circumflex artery (Cx) also demonstrated significant dilation, but without stenosis (Figure 1). In light of this situation, coronary surgery was performed (left internal mammary artery to LAD and right internal mammary artery to RCA). Intraoperative images show the RCA (Figure 2A) and the proximal portion of the LAD (Figure 2B). The postoperative progress was favorable and there were no adverse events. Figure 2. RCA

Pressure necrosis of the outlet septum caused by two adjacent implants.

A patient had a history of a Ross-Konno procedure, stenting of the pulmonary homograft, and an aortic valve replacement. Pressure necrosis developed in the tissues in between the stent and the prosthetic valve. This resulted in a defect between the right and left ventricular outflow tracts that required a complex operation for its correction.