Cardiac lymphatics

Cardiac lymphatics in pigs (Deutsche Landrasse pigs):

Image from Vazques-Jimenez, JF. et al. Cannulation of the cardiac lymphatic system in swine. Eur J Cardiothorac Surg 2000;18:228-32. Reprint provided by Dr. M. Thapar. Read also the letters to the editor on this article.

Bicuspid Aortic Valve - Etiology and Associated Lesions

The following letter to the editor highlights other articles that deal with different etiologies for different morphologies of bicuspid aortic valve. Worth reading them all!

J Am Coll Cardiol, 2010; 56:1680, doi:10.1016/j.jacc.2010.03.073 Bicuspid Aortic Valve Morphology

Alexander R. Opotowsky, MD, MPH^_* andMichael J. Landzberg, MD

^_* Children's Hospital Boston, Boston Adult Congenital Heart Service, Department of Cardiology, 300 Longwood Avenue, Boston, Massachusetts 02115 (Email:alexander.opotowsky@childrens.harvard.edu).

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We read with interest and appreciation 3 recent papers in the Journal on the bicuspid aortic valve (BAV).

Fernandez et al. (1) describe distinct developmental patterns for mice and hamsters with right-noncoronary and right-left coronary cusp fusion, respectively. Incredibly, William Osler anticipated, within the limitations of his era, these findings and their significance more than a century ago:

If it turns out to be correct ... that the affected valves are usually those behind ... the coronary arteries ... this would point to some error associated especially with the development of these cusps. It would appear from the observations of Tonge, that two of the segments are formed before the division of the primitive truncus arteriosus is complete, while the third arises laterafter the pulmonary artery and the aorta have divided. It is not at all improbable that we may have here a clew to an explanation of this anomaly, but this is conjectural until we have fullerdetails of the process of the development of the sigmoid valves in mammals (2).

As it becomes increasingly apparent that right-noncoronary and right-left coronary cusp fusion are distinct diseases, research reports on the BAV should make this distinction as Osler suggested: "This point [right-left coronary cusp fusion is the most common BAV morphology], previously overlooked, may prove of interest in the etiology, and should be carefully noted in future observations" (2).

Biner et al. (3) report evidence of a bicuspid aortopathy in first-degree relatives of BAV patients, but did not address the relationship of BAV morphology to aortic properties. We would be interested to know whether BAV morphology in the proband modifies the extent of aortic dilation and stiffness in first-degree relatives.

Tzemos et al. (4) provide data suggesting that the BAV is associated with endothelial dysfunction, at least in the presence of aortic dilation. The authors note that three-fourths of the patients in each BAV group had anteroposterior aortic leaflet orientation (presumably right-left coronary cusp fusion), but no data arepresented on the relationship between BAV morphology and the parameters studied. Does BAV morphology influence the relationship among aortic dilation, aortic stiffness, serum matrix metalloproteinaselevels, and endothelial function?

With deepening understanding of the developmental and physiologic aspects of the BAV and its associated diffuse vasculopathy, we believe that it is vital that data be reported to allow detailed inquiry into potential variation between morphologically and likely clinically and developmentally distinct categories of disease.

References:

1. Fernandez B, Duran AC, Fernandez-Gallego T, et al. Biscupid aortic valves with different spatial orientations of the leaflets are distinct etiological entities J Am Coll Cardiol 2009;54:2312-2318.[Abstract/Free Full Text]

2. Osler W. The Bicuspid Condition of the Aortic Valves. . Transactions of the Association of American Physicians. Philadelphia: Wm. J. Dornan; 1886. pp. 185-192.

3. Biner S, Rafique AM, et al. Aortopathy is prevalent in relatives of bicuspid aortic valve patients J Am Coll Cardiol 2009;53:2288-2295.[Abstract/Free Full Text]

4. Tzemos N, Lyseggen E, Silversides C, et al. Endothelial function, carotid-femoral stiffness, and plasma matrix metalloproteinase-2 in men with bicuspid aortic valve and dilated aorta J Am Coll Cardiol 2010;55:660-668.[Abstract/Free Full Text]

Also see other posting on this subject

Fernandez et al (ref#1 above)

Echo: 3D imaging of ASD & Right Atrium

Anatomy of Right Atrial Structures by Real-Time 3D Transesophageal Echocardiography
Francesco F. Faletra, Siew Y. Ho, and Angelo Auricchio
J Am Coll Cardiol Img 2010;3 966-975

3D Echocardiography of the Atrial Septum: Anatomical Features and Landmarks for the Echocardiographer
Kuberan Pushparajah, Owen I. Miller, and John M. Simpson
J Am Coll Cardiol Img 2010;3 981-984

Surgery: Pulmonary atresia - VSD, MAPCAs, Unifocalization

Circulation. 2000 Apr 18;101(15):1826-32.
Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients.
Reddy VM, McElhinney DB, Amin Z, Moore P, Parry AJ, Teitel DF, Hanley FL.
Divisions of Cardiothoracic Surgery, University of California, San Francisco 94143-0118, USA.

BACKGROUND: Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients.


METHODS AND RESULTS: Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients.

CONCLUSIONS: Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.




MAPCAs arrangement in 11 patients with PA-VSD reported in JTCVS 1997 paper from Quebec:
J Thorac Cardiovasc Surg 1997;114:727-737. One-satge midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aorto-pulmonary collaterals. Christo I. Tchervenkov et al.

Group I (pt #1-6) had multi-stage repair:
Group II (pt #7-11) had single-stage repair:
Also see other postings on this subject:
Pre-op evaluation of PAs and MAPCAs
MAPCAs in PA-VSD
Surgery Algorithms for PA-VSD